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Haematological Parameters of Adult and Paediatric Subjects with Sickle Cell Disease in Steady State, in Benin City, Nigeria

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... Global Youth Tobacco Survey indicated that little difference existed between the genders in cigarette smoking or in use of other tobacco products. From 120 sites that collected data on cigarette smoking by boys and girls, more than one-half (n = 61) showed no difference by 21 gender. ...
... In concordance with other studies, there was no statistically significant association between 1 0mean PLT and age in this study. 12,15,21 However, we observed a gender-based difference in mean PLT, males had a higher mean PLT than females. This finding is at odds 22 with the widely held view. ...
... The reasons for this finding in this cohort is not evident, nonetheless it may be due to the fact that the majority of our female participants were prepubertal, limiting the effects of variations in hormonal profiles, as well as that of menstruation induced compensatory haematopoiesis on PLT. Our findings in this contextwere however, similar to that of 21 Iheanacho in Benin City, Nigeria. ...
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Background: Nigeria has the highest burden of sickle cell disease (SCD) in the world, yet the vast majority of its patients do not have access to evidence-based cure. Haematological parameters in steady-state remain useful as indicators for initiating treatment and monitoring response to therapy as well as for prognostication. Objectives:This study aimed to determine the steady- state haematological parameters of children with SCD at a reference hospital in North-eastern Nigeria. Methods: A prospective observational study of children with steady state sickle cell disease, and aged 2 years – 16 years, was conducted from January, 2019 – October, 2020. The following haematological indices were obtained from participants; total leucocyte count, platelet count, haematocrit and haemoglobin concentration. Analysis of data was done with the statistical package for social sciences (SPSS) version 20.0. Results: One hundred patients were recruited. The mean age was 6.97 ± 3.63 years, with 61(61%) males and 39(39%) females giving a male: female ratio of 1.6:1. Mean total leucocyte count, mean platelet count, mean haemoglobin concentration and mean haematocrit were16.83±7.30 x 109/L, 356.66±178.27x109/L, 7.65±1.45g/dL and 23.51±3.90% respectively. Males had a significantly higher mean platelet count than females (P= 0.02). However, gender-based differences in the other haematological parameters were not statistically significant. Conclusion: The present study found raised levels of total leucocyte counts and platelet counts but low haematocrit and haemoglobin concentrations in children with steady- state SCD. These findings would be helpful in the management of paediatric SCD in this part of Nigeria. KEYWORDS: Sickle cell disease; Total leucocyte count; Platelet count; Haematocrit; Haemoglobin concentration.
... Severe autosplenectomy in this study is far less than 55.4% reported among Northern Nigerian patients with SCA and 33.3% of patients in Turkey but more than the 6.6-15.5% reported among Eastern Saudi Arabian patients 18,20,21 . Splenomegaly in SCA is unusual after the first decade of life, however, in this current study, 10% of patients had splenomegaly which is lower the 35.3% documented in paediatric patients 22 . This may be due to relatively older population in our study. ...
... Comparing haematological parameters in patients with SCA and normal HbA controls, the Hb and Hct of patients are significantly less than that of non SCA while the white cells and platelet counts are higher. This findings corroborates previous studies 13,22 . ...
... In comparison, the reports on red cell indices are rather inconsistent. While Akinbami 24 and Omoti 25 both from different parts of Nigeria reported a lower MHC, MCV and MCHC in patient than in the controls, these indices were comparable for both patients and controls in our study as well as some other studies from Nigeria by Iheanacheo 22 . The explanation for the lower values in patients compared to controls in the studies were anaemia of chronic disease, infections and haemolysis. ...
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Background: The spleen serves critical haematological and immunological functions in the body. However it is also the first organ to be affected by the effects of sickling in sickle cell anaemia. While the splenic size has been evaluated in sickle cell anaemia, the spleen sizes of these patients has not been associated with any specific haematological pattern. Objectives: To determine the haematological parameters of patients with sickle cell anaemia (SCA) in relation to spleen size. Methods: The full blood count (FBC), the irreversibly sickled cells and blood transfusion use amongst SCA patients in steady state was evaluated. Abdominal ultrasound was also performed for all patients and HbAA control for splenic size categorization. Results: Forty patients with SCA and 22 controls with HbAA were studied with mean age of 29.28 ± 8.10 years 28.23 ± 8.14 years respectively. The mean splenic longitudinal lengths in patients and controls were 6.3 ± 4.3cm and 8.9 ± 1.5 cm respectively (p-value < .05). The mean haematocrit and haemoglobin value were significantly lower in SCA cases than in controls (p < 0.001). Though the red cell indices were similar but the white blood cell and platelet count were significantly higher in patients than in controls. Among SCA cases, the spleen size showed significant positive correlation with haematocrit (r = 0.371, p = 0.019) and the age at 1st transfusion (r = 0.447, p = 0.013) but significant negative correlation with MCV, MCH, MCHC and platelet count. Above 80% of patients with severe, moderate and mild autosplenectomy had been transfused compared to 25% of patients with splenomegaly. Conclusion: Similarities in red cell indices between patients and controls suggests an identical factor influencing the red cell indices which could be genetic such as thalassemia or environmental such as iron deficiency. The negative correlation of the spleen size with red cell indices, white cell count and platelet count and positive correlation with haematocrit suggest that spleen size can be used to determine clinical course of the disease. Earlier age at first transfusion, significantly higher frequency of blood transfusion and MCHC in patients with severe autosplenectomy suggest a more severe clinical course when compared with patients with splenomegaly, normal spleen, mild and moderate autosplenomegaly.
... Curative options, such as bone marrow transplantation, are becoming increasingly available although challenged by the high risk of complications with the procedure and, in lowincome countries, high cost, lack of expertise, and lack of facilities [6,7]. SCD primarily affects red blood cells but also causes changes in other hematological components such as white blood cells and platelets [8,9]. Individuals with SCD go through alternating periods of stable health (steady state) and crises associated with complications such as anemia and pain [10]. ...
... Overall, most of the hematological and biochemical parameters in individuals with SCD are different from those of the general population [8, 9,13,14]. Thus, the significance of laboratory reference values for SCD has received considerable attention in recent years [8,9,[13][14][15]. ...
... Overall, most of the hematological and biochemical parameters in individuals with SCD are different from those of the general population [8, 9,13,14]. Thus, the significance of laboratory reference values for SCD has received considerable attention in recent years [8,9,[13][14][15]. Unfortunately, there is a scarcity of reliable data to serve as reference values for hematological and biochemical parameters among patients with SCD in most African countries [16][17][18]. ...
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Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and the evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, Tanzania, to establish laboratory reference ranges among children and adults with SCD at steady state. Patients were grouped into five age groups and according to their sex. Aggregate functions were used to handle repeated measurements within the individual level in each age group. A nonparametric approach was used to smooth the curves, and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4422 patients collected from 2004–2015 were analyzed. The majority of the patients (35.41%) were children aged between 5–11 years. There were no significant differences (p ≥ 0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils, and direct bilirubin observed between males and females. Significant differences (p < 0.05) were observed in all selected parameters across age groups except with neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when the SCD estimates were compared to the general population. The laboratory reference ranges in SCD at steady state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady state will be helpful in the management and monitoring of the progress of SCD.
... Curative options, such as bone marrow transplantation, are becoming increasingly available although challenged by the high risk of complications with the procedure and, in lowincome countries, high cost, lack of expertise, and lack of facilities [6,7]. SCD primarily affects red blood cells but also causes changes in other hematological components such as white blood cells and platelets [8,9]. Individuals with SCD go through alternating periods of stable health (steady state) and crises associated with complications such as anemia and pain [10]. ...
... Overall, most of the hematological and biochemical parameters in individuals with SCD are different from those of the general population [8, 9,13,14]. Thus, the significance of laboratory reference values for SCD has received considerable attention in recent years [8,9,[13][14][15]. ...
... Overall, most of the hematological and biochemical parameters in individuals with SCD are different from those of the general population [8, 9,13,14]. Thus, the significance of laboratory reference values for SCD has received considerable attention in recent years [8,9,[13][14][15]. Unfortunately, there is a scarcity of reliable data to serve as reference values for hematological and biochemical parameters among patients with SCD in most African countries [16][17][18]. ...
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Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and evaluation of interventions. This study was conducted at Muhimbili National Hospital (MNH) in Dar es Salaam, to establish laboratory reference ranges in SCD at steady-state. Patients were grouped into five age groups with respects to their sex. Aggregate functions were used to handle repeated measures within the indi-vidual level in each age group. A nonparametric approach was used to smooth the curves and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4,422 patients collected from 2004-2015 were analyzed. The majority of the patients (35.41%) were children aged between 5-11 years. There were no significant differences (p≥0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils and bilirubin direct observed between males and females. Significant differences (p<0.05) were observed in all selected parameters across age groups except neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when SCD estimates were compared to the general population. Laboratory reference ranges in SCD at steady-state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady-state will be a helpful in the management and monitoring of the progress of SCD.
... Bone marrow transplantation of hematopoietic stem cells has been shown to cure SCD despite the complication of the procedures, high costs and limited facilities that are available mainly in high-income countries [5,6]. SCD primarily affects red blood cells but also causes changes in other hematological parameters like white blood cells and platelets [7,8]. Individuals with SCD go through periods characterized by steady-state and crises which associated with sickle cell complications such as anemia and pain [9]. ...
... Furthermore, research has shown that patients with high leukocyte count, low steady-state hemoglobin and lower fetal hemoglobin level (HbF) have increased risks for sickle cell crises, including stroke [10]. Although there is a scarcity of hematological and biochemical parameters in most African countries [11][12][13], the significance of these laboratory reference values for SCD has received considerable attention in recent years [7,8,[14][15][16]. Reference values are widely used in medical decision-making. ...
... The findings of 7.2-7.3g/dL hemoglobin concentrations in SCD children of under-five years were higher than 6.3-6.9g/dL reported by other researchers [8,14,23] ; but lower than 8.1-9.3g/dL which have been reported in the cooperative study of sickle cell disease conducted in Western countries [24]. ...
Preprint
Full-text available
Hematological and biochemical reference values in sickle cell disease (SCD) are crucial for patient management and evaluation of interventions. This study was conducted at Mu-himbili National Hospital (MNH) in Dar es Salaam, to establish laboratory reference ranges in SCD at steady-state. Patients were grouped into five age groups with respects to their sex. Aggregate functions were used to handle repeated measures within the indi-vidual level in each age group. A nonparametric approach was used to smooth the curves and a parametric approach was used to determine SCD normal ranges. Comparison between males and females and against the general population was documented. Data from 4,422 patients collected from 2004-2015 were analyzed. The majority of the patients (35.41%) were children aged between 5-11 years. There were no significant differences (p≥0.05) in mean corpuscular hemoglobin concentration (MCHC), lymphocytes, basophils and bilirubin direct observed between males and females. Significant differences (p<0.05) were observed in all selected parameters across age groups except neutrophils and MCHC in adults, as well as platelets and alkaline phosphatase in infants when SCD estimates were compared to the general population. Laboratory reference ranges in SCD at steady-state were different from those of the general population and varied with sex and age. The established reference ranges for SCD at steady-state will be a helpful in the management and monitoring of the progress of SCD.
... In this study, leucocytosis (elevated TLC) was a characteristic finding in children with SCA; it was seen in more than two-thirds of the subjects. This finding is similar to the reports of other studies [7,8]. The leucocytosis in SCA at steady state has been hypothesised to be a reflection of the constant inapparent inflammation with consequent cytokine release ultimately leading to increased leucocyte production in the bone marrow [9]. ...
... In this study, the mean leucocytosis was significantly higher in male subjects compared to females. This is similar to findings by Abubakar et al. and Iheanacho et al. who have also reported higher TLC values in male subjects with SCA [7,8]. Elevated platelet count (thrombocytosis) was seen in one-third of the children with SCA. ...
... Elevated platelet count (thrombocytosis) was seen in one-third of the children with SCA. This is consistent with findings relating to thrombocytosis that have been reported by other similar studies [7,8,13]. Thrombocytosis in SCA has been variably attributed to anaemia-induced increased erythropoietin secretion that has similar homology to thrombopoietin with consequent thrombopoiesis and decreased pooling of platelets in splenic circulation due to functional and/or structural asplenia characteristic of SCA [14,15]. ...
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Introduction Sickle cell anaemia (SCA) is an inherited, autosomal recessive condition that results from a mutation in the β-globin gene. Vascular occlusion is the underlying mechanism behind a myriad of complications encountered. This vascular occlusion is primarily caused by the increased tendency of red blood cells (RBC) to adhere to the vascular endothelium, and the activation of platelets and total leucocyte count (TLC), hence the need for a steady-state haematological profile in these patients. Method This was a cross-sectional study conducted over four months at a sickle cell clinic. Haemoglobin (Hb) concentration, haematocrit, platelet, TLC, mean corpuscular volume (MCV), mean corpuscular haemoglobin (MCH) and mean corpuscular haemoglobin concentration (MCHC) of the subjects were recorded and analysed. Results Ninety-nine subjects aged 1-18 years were recruited for the study. There were 53 (53.5%) males. Leucocytosis was seen in 80 (80.8%), anaemia in 99 (100%), and thrombocytosis in 30 (30.3%) patients. The mean Hb, TLC and platelets were 7.9 ± 1.3g/dl, 14.3 ± 4.5 x 103/mm3 and 391.5 ± 182.6 x 103/mm3 respectively. Mean MCV, MCH and MCHC were 81.3 ± 7.1 fl, 28.6 ± 2.9 pg and 35.2 ± 1.7 g/dl respectively. Children aged one to four years had the highest TLC (p=0.002) but the lowest mean Hb and platelet (p=0.094 and 0.06) respectively. The mean MCV, MCH and MCHC were lowest in children aged one to four years (p=0.047, 0.001 and 0.001). Conclusion Anaemia, leucocytosis and thrombocytosis are characteristics features of children with SCA, especially in male and younger subjects. Although Iron markers are generally normal in children with SCA, those under the age of five years tend to have lower values.
... Sickle Cell Anaemia (SCA) is majorly a Red Blood Cell (RBCs) disorder with significant changes observed in certain haematological parameters [1]. This commonest hereditary disorder is characterized by severe haemolytic anaemia with different haematological changes and is also considered as a chronic inflammatory disease [2]. ...
... [9]. Other parameters for assessment include the red cell indices such mean cell volume, mean cell haemoglobin and mean cell haemoglobin concentration which is useful in detecting co-exiting causes of anaemia in the patients [1]. Although there are no readily available cures for SCA in developing nations, the World Health Organization has identified that most of the mortalities caused by SCA and its complications can be prevented through proper monitoring [10]. ...
... High WBC count (≥ 11 x 109 /L) is associated with SCD complications including cerebrovascular accidents [12], [15]. Other parameters for assessment include the red cell indices which are useful in detecting co-existing causes of anaemia in the patients [16]. In the past two decades, several modalities of treatment of SCD such as blood transfusion and haematinic have been used and these modify the haematological indices of SCD patients. ...
... The mean platelet count of the VOC SCD patients was lower than that of SCD patients in steady state. This could be due to the decrease or absent splenic sequestration of platelets in SCA patients in steady state [16]. These findings are in consonance with several other reports [8], [20], [21]. ...
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Background: Sickle cell disease (SCD) is a hereditary haemoglobinopathy characterized by deranged haematopoiesis, and intermittent occlusion of small blood vessels. Patients can be in relative good health " steady state " which may be punctuated by acute exacerbations called " crisis " believed to be precipitated by factors that may affect their haematological indices. Methods: This prospective study involved 100 subjects consecutively recruited from haematology day care clinics of the University of Maiduguri Teaching Hospital. This comprised 50 sickle cell anaemia patients (HbS HbS) in vaso-occlusive crisis and 50 sickle cell anaemia patients (HbS HbS) in steady state. EDTA anticoagulated blood samples were collected from all subjects for the determination of packed cell volume, white blood cell counts, platelet counts and differential leukocyte counts using standard methods. Results: The mean age ± standard deviations of the subjects in steady state and in vaso-occlusive crisis are 23.94±7.14 and 23.60±6.28. The packed cell volume of patients in steady state and those in crisis are 0.18±0.05 and 0.17±0.04, respectively (p =0.58); the white blood counts of patients in steady state and those in crisis are 14.21±4.27 and 14.56±4.77, respectively (p-value 0.70) and the platelet counts of patients in steady state and those in crisis are 121.44±3.61 and 110.14±3.81, respectively with (p =0.21). The differential leukocyte counts of patients in steady state versus those in crisis are neutrophils. There is no significant statistical difference (p˃0.05) in the mean values of PCV, WBC count, Platelet count and differential white cell count between sickle cell patients in steady state and during vaso-occlusive crisis. Conclusion: Findings from this study showed that there is no difference in the haematological parameters of sickle cell anaemic patients in crisis state from those in stable state. However, detailed red cell indices could provide baseline data that would be used in effective evidenced-based management of sickle cell diseases.
... Even with the low hemoglobin levels, SCA patients maintain normal function and activity with their steadystate level, only requiring blood transfusion during intercurrent illnesses and acute crises resulting in acute fall in Hb. High total WBC, differential, and platelet count demonstrated by a previous study in SCA agree with this current study [28] Asplenia with loss of splenic pool, preferential increase in marginating pool, and increased inflammatory cytokines during vaso-occlusive events increases platelet and WBC counts in SCA which impacts on SCA morbidity and mortality. The mean platelet count of 369 x 109 in our patients was higher than reported by Iheanacho et al. [28]. ...
... High total WBC, differential, and platelet count demonstrated by a previous study in SCA agree with this current study [28] Asplenia with loss of splenic pool, preferential increase in marginating pool, and increased inflammatory cytokines during vaso-occlusive events increases platelet and WBC counts in SCA which impacts on SCA morbidity and mortality. The mean platelet count of 369 x 109 in our patients was higher than reported by Iheanacho et al. [28]. The patients with SCA in the current study did not benefit from the cytoreductive effect of hydroxyurea therapy due to financial constraints [29]. ...
Article
Introduction: nutritional status is an under-studied environmental factor that can impact the phenotypic manifestations of patients with Sickle Cell Anaemia (SCA). This study aimed to define hemato-biochemical parameters and micronutrient status in patients with SCA. Methods: this was a cross-sectional study of patients with SCA and hemoglobin genotype HBAA controls at a tertiary health facility in Abakaliki, from 2nd December 2020 to 31st March 2021. Plasma micronutrient levels, haemato-biochemical parameters were analyzed and anthropometric measurements obtained from all participants. Results: sixty participants with SCA had 58.3% females (mean age of 24.77±7.39 years) while controls had 50% females (mean age of 26.23 ± 8.44 years). The SCA group had significantly lowered calcium (2.733 ± 1.593 vs 1.846 ± 1.123 mmol/l; p=0.009) and magnesium (19.38 ± 6.37 vs 9.65 ± 1.38 mg/dl; p= < 0.001) levels but higher plasma iron (1.70 ± 0.89 vs 1.06 ± 0.53; p=0.001). Zinc and Copper did not reveal significant differences between the two groups. Chloride ion levels was significantly lower in the SCA patients (107.50 ± 17.42 vs 100.19 ± 12.92; p=0.026) while Alkaline phosphatase (ALP), bilirubin, total white blood cell (WBC) and platelets (PLT) count were higher compared with the HBAA group (255.72 ± 124.52 vs 134.56 ± 39.67; p= <0.001, 46.86 ± 25.03 vs 25.63 ± 18.80; p = 0.001, 13.21± 6.57 vs 6.10 ± 1.35; p= < 0.001 and 369.25 ± 138.11 vs 209.36 ± 47.85; p= <0.001). Conclusion: Copper and zinc deficiency was not present in our population of SCA patients but, they had lower plasma calcium and magnesium levels and elevated levels of blilirubin, ALP, WBC and platelets PLT counts. These parameters can be explored in designing better management for patients with SCA.
... Sickle Cell Anaemia (SCA) is majorly a Red Blood Cell (RBCs) disorder with significant changes observed in certain haematological parameters [1]. This commonest hereditary disorder is characterized by severe haemolytic anaemia with different haematological changes and is also considered as a chronic inflammatory disease [2]. ...
... [9]. Other parameters for assessment include the red cell indices such mean cell volume, mean cell haemoglobin and mean cell haemoglobin concentration which is useful in detecting co-exiting causes of anaemia in the patients [1]. Although there are no readily available cures for SCA in developing nations, the World Health Organization has identified that most of the mortalities caused by SCA and its complications can be prevented through proper monitoring [10]. ...
... 30 Total Hb level vary in SCA patients according to the βS haplotypes. 18 The lower levels of Hb and HCT among SCD patients in the current study agree with the work of Tshilolo et al 31 and Omoti. 2 Chronic hemolysis, 32 shortened red cell survival, 33 as well as low erythropoietin response 34 associated with SCD might have been involved in the reduced Hb and HCT levels observed in the present study. The Hb levels in this study are similar to the finding of Nagose and Rathod. ...
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Background: Effective treatment and management of sickle cell disease (SCD) has been a challenge in Africa over the years. Hematological parameters are very useful profiles in the effective management of the disease. However, there is scarcity of studies on the hematological parameters of SCD in Ghana. This study aimed at determining hematological parameters among SCD patients with vaso-occlusion, those in the steady state as well as healthy controls at a teaching hospital in Ghana. Methodology: This was a cross-sectional study involving a total of 628 subjects, including 148 HbAA controls, 208 HbSS patients in steady state, 82 HbSC patients in steady state, 156 HbSS patients in vaso-occlusive crises (VOC), and 34 HbSC patients in VOC. Venous blood sample was collected from all study participants. A full blood count was done within 2 hours of collection, and hemoglobin (Hb) concentration, packed cell volume, red blood cell (RBC) concentration, mean corpuscular Hb, mean cell volume, mean corpuscular Hb concentration, and white blood cells (WBC) and platelet (PLT) counts were recorded. Results: WBC and PLT counts were significantly higher in both female and male patients with SCD, compared with their healthy counterparts (P<0.05). The level of WBC was, however, significantly higher in patients with HbSS VOC among the SCD patients (P<0.001). Levels of Hb, RBC, and hematocrit were significantly higher in the controls (P<0.001). There was no significant difference in mean cell Hb among male patients with SCD (P=0.274) and female patients with SCD (P=0.5410). Conclusion: The SCD patients had lower Hb and RBC than the controls; however, higher PLT and WBC are noted in various status of SCD, possibly reflecting spleen effect in these patients. Further studies are needed to confirm these findings.
... Our study showed that majority of the participants were aged 5 years and above, with 80% in steady, 50% in VOC. The age group of our cohort of SCA subjects in this study are similar to subjects studied in previous reports [23][24][25][26]. Life expectancy of SCA patients are reduced considerably among younger children and older people. ...
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Abstract Introduction: Sickle cell disease (SCD) is a major public health challenge particularly in sub Saharan Africa. It is one of the most common genetic diseases in the world. Objectives: The aim of this study was to investigate the socio-demographic distribution of children with sickle cell disease presenting to the Paediatric Department of Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. Materials and method: The current study included 60 consecutively recruited children with SCD [30 in vaso-occlusive crisis (VOC) and 30 on steady state] aged 1 - 14 years who were admitted to UDUTH. Socio-demographic characteristics of the children were collected using a questionnaire. Results: Result from this study indicated that majority of the participants were aged 5 years. Gender distribution indicated an equal distribution of males and females (50%) among subjects in steady state group but a slight increase of females than males in VOC group (56.7%). Ethnic distribution indicated that a significant number of the SCA subjects (steady and VOC) were of the Hausa/Fulani ethnic group (90%). Distribution based on educational status of paternal parents indicated that a significant number of fathers of subjects in the steady patient were educated up to the tertiary level (56.7%) while majority of the paternal parents of children in vaso-occlusive crisis were educated up to secondary school (36.7%). Most of the maternal parents were either secondary school leavers (40.0% for steady, 33.3% for VOC) or had no formal education (23.3% for steady and 33.3% for crisis). Distribution of the SCD subjects based of school attendance indicated that majority of the subjects in the steady state (73.3%) attend school while a significant 53.3% of those in VOC did not attend school (53.3%). In terms of position of the child in the family, most of SCD subjects in the steady state were in position 1 - 5 (66.7%) while majority of the subject in VOC were (46.7%) were ≥ 6th position. In terms of the number of wives in the family, most of SCD subjects in the steady state (40%) and VOC (43.3%) were from monogamous families. Distribution of the subjects based on the occupation of the fathers indicated that majority of the fathers of the subjects in the steady (53.3%) and VOC (53.3%) were business men. Distribution of the subjects based on the occupation of the mothers indicated that majority of the mothers of the subjects in the steady (66.7%) and VOC (76.7%) were farmers. Distribution of the subjects based on the income of the fathers indicated that majority of the fathers of the subjects in the steady state (50%) earned > 100,000 naira while majority of the fathers of subjects with VOC (43.3%) earned < 18,000 naira. Distribution of the subjects based on the income of the mothers indicated that majority of the mothers of the subjects in the steady state (53.5%) and VOC (83.3%) earned < 18,000 naira. Distribution based on presence of plasmodium parasitaemia indicated that 23.3% of SCD subjects in the steady state had malaria infection compared to 76.7% among those with VOC. Distribution based on presence of infection indicated that 36.7% of SCD subjects in the steady state had infection compared to 60% among those with VOC. Conclusion: This study confirms that sociodemographic variables play a role in hospitalization of SCA children to hospital. Sociodemographic characteristics should be considered seriously in the management of SCD patients. Carrier detection and genetic counselling through antenatal and newborn screening, couple/premarital screening, and other forms of population screening should be implemented. There is need for the provision of a holistic care that takes into consideration the sociodemographic .
... Interestingly, a subset of this cohort showed altered expression of inflammatory cytokines, including CXCL10, TNF-a, CCL2, IL-8, and IL-6, associated with hemoglobin genotypes and P. falciparum infections [18]. The increased PLT counts in HbSS-were significantly higher (p < 0¢05) than observed for all the other genotypes, due to reduced splenic sequestration of PLT and increased erythropoietin associated with SCA (Fig. 1D) [19,20]. ...
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Background: Sickle cell anemia (SCA) is a severe monogenic disorder, caused by single nucleotide mutations in the hemoglobin (Hb) gene, that is prevalent in malaria endemic regions of the world. Sickle cell trait (SCT) individuals carry only one of the mutated alleles and were shown to be protected against malaria. However, defining the relative contribution of hematological, clinical, and environmental factors to the overall burden of malaria in individuals with hemoglobinopathies such as SCA has been challenging. Methods: We hypothesized that hematological differences, clinical presentations, and self-reported bed net usage among Plasmodium-infected and uninfected individuals may govern overall malaria burden in individuals with sickle cell disease (SCD). We conducted a cross-sectional study in Ghana from 2014 to 2019 and described clinical presentations, hematological characteristics, and bed net use based on a comprehensive questionnaire. Hematological characteristics were compared using a parametric or nonparametric ANOVA, pending if data passed D'Agostino & Pearson normality test. When comparing only two Hb genotypes hematological characteristics a Mann-Whitney U-test were used. Logistic regressions and Chi-squared tests were used to compare questionnaire responses between Hb genotypes. All statistical significance was set at p < 0.05. Findings: Multiple hematological parameters were significantly (p < 0.05) altered depending on sickle cell genotype and/or malaria status. When compared to other Hb genotypes, SCA individuals with or without malaria had significantly (p < 0.05) higher WBC and platelets counts and lower Hb levels. While the sickle cell genotype may affect malaria severity, SCT and SCA participants were found to significantly (p < 0.007) use bet nets more than HbAA participants. Interpretations: Our findings can be utilized to enhance national guidelines for reducing the incidence of malaria especially among individuals with SCD, SCT protection and health disparities among hemoglobinopathies. Funding: This study was supported by the National Institute for Health.
... In our research also, it was observed that the Hb SS group possessed significantly higher platelet and total white blood cell counts when compared to the other Hb groups. This finding is consistent with other previous studies which linked white cell and platelet counts with disease severity in sickle cell disease [40,41]. Current patho-physiologic mechanism of vaso-occlusion in sickle cell disease has been found to involve the white cells and platelets as well as the expression of their intercellular adhesion molecules [42,43]. ...
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In view of the association of various haemoglobin electrophoretic patterns with different pathological conditions and obesity with its attendant risk factors for cardiac and pulmonary disorders, the present study evaluated the relationship between body mass index and haematological indices by randomly selecting young adult Nigerians with different haemoglobin electrophoretic patterns within the age group of 17-45 years and mean age of ±31 years old. 215 participants were enlisted for this study with their BMI and other anthropometric indices measured and grouped into different BMI categories as recommended by the World Health Organization. Haematological indices such as packed cell volume, total and differential white blood cell count, and platelets as well as haemoglobin (Hb) electrophoresis were assessed in relation to their anthropometric measurements using standard methods. We observed a significantly increased neutrophil and platelet counts in the subjects with BMI > 25 kg/m 2. BMI was also observed to be positively correlated with the neutrophil, monocyte counts and MCV of haemoglobin AS and SS genotype groups in this study. This study showed a higher percentage of overweight and obesity among females, and haematological dyscrasias in mostly the HbSS subjects. Knowledge of the relationship between BMI and hematological indices of apparently healthy individuals within any population is therefore essential in healthcare planning, as a justification for early prognosis and genetic counselling policy strategically reducing the incidence of obesity, its attendant conditions and haemoglobinopathies in Nigeria.
... Our study showed that majority of the participants were aged 5 years and above, with 80% in steady, 50% in VOC. The age group of our cohort of SCA subjects in this study are similar to subjects studied in previous reports [23][24][25][26]. Life expectancy of SCA patients are reduced considerably among younger children and older people. ...
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Introduction: Sickle cell disease (SCD) is a major public health challenge particularly in sub Saharan Africa. It is one of the most common genetic diseases in the world. Objectives: The aim of this study was to investigate the socio-demographic distribution of children with sickle cell disease presenting to the Paediatric Department of Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. Materials and method: The current study included 60 consecutively recruited children with SCD [30 in vaso-occlusive crisis (VOC) and 30 on steady state] aged 1 - 14 years who were admitted to UDUTH. Socio-demographic characteristics of the children were collected using a questionnaire. Results: Result from this study indicated that majority of the participants were aged 5 years. Gender distribution indicated an equal distribution of males and females (50%) among subjects in steady state group but a slight increase of females than males in VOC group (56.7%). Ethnic distribution indicated that a significant number of the SCA subjects (steady and VOC) were of the Hausa/Fulani ethnic group (90%). Distribution based on educational status of paternal parents indicated that a significant number of fathers of subjects in the steady patient were educated up to the tertiary level (56.7%) while majority of the paternal parents of children in vaso-occlusive crisis were educated up to secondary school (36.7%). Most of the maternal parents were either secondary school leavers (40.0% for steady, 33.3% for VOC) or had no formal education (23.3% for steady and 33.3% for crisis). Distribution of the SCD subjects based of school attendance indicated that majority of the subjects in the steady state (73.3%) attend school while a significant 53.3% of those in VOC did not attend school (53.3%). In terms of position of the child in the family, most of SCD subjects in the steady state were in position 1 - 5 (66.7%) while majority of the subject in VOC were (46.7%) were ≥ 6th position. In terms of the number of wives in the family, most of SCD subjects in the steady state (40%) and VOC (43.3%) were from monogamous families. Distribution of the subjects based on the occupation of the fathers indicated that majority of the fathers of the subjects in the steady (53.3%) and VOC (53.3%) were business men. Distribution of the subjects based on the occupation of the mothers indicated that majority of the mothers of the subjects in the steady (66.7%) and VOC (76.7%) were farmers. Distribution of the subjects based on the income of the fathers indicated that majority of the fathers of the subjects in the steady state (50%) earned > 100,000 naira while majority of the fathers of subjects with VOC (43.3%) earned < 18,000 naira. Distribution of the subjects based on the income of the mothers indicated that majority of the mothers of the subjects in the steady state (53.5%) and VOC (83.3%) earned < 18,000 naira. Distribution based on presence of plasmodium parasitaemia indicated that 23.3% of SCD subjects in the steady state had malaria infection compared to 76.7% among those with VOC. Distribution based on presence of infection indicated that 36.7% of SCD subjects in the steady state had infection compared to 60% among those with VOC. Conclusion: This study confirms that sociodemographic variables play a role in hospitalization of SCA children to hospital. Socio-demographic characteristics should be considered seriously in the management of SCD patients. Carrier detection and genetic counselling through antenatal and newborn screening, couple/premarital screening, and other forms of population screening should be implemented. There is need for the provision of a holistic care that takes into consideration the sociodemographic status of the patient in a bid to ensuring optimum outcomes and to reduce SCD -related hospitalizations and mortality.Keywords: Socio-Demography; Children; Sickle Cell Disease; Usmanu Danfodiyo University Teaching Hospital; Sokoto; Nigeria.
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Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world. It is associated with oxidative stress which occurs as a result of HbS unstable character causing a rise in the formation of free radicals. The aim of this study was to determine some antioxidant enzymes activities among patients with SCD. We investigated the superoxide dismutase (SOD), and glutathione peroxidases (GPx) levels among 60 children aged 1 - 14 years with SCD. Twenty-two age-matched non-SCD children served as control. The study subjects were divided into two groups; steady state A (n = 30) and vaso- occlusive crisis (VOC) B (n = 30). The SOD, and GPx levels were significantly lower among the SCD subjects compared to controls (p = 0.000). There were no statistically significant differences in the SOD and GPX levels between sickle cell disease patient in steady state (A) and those in crisis (B) (p = 0.998 and 0.555) respectively. There was a statistically significant difference between the SOD and GPX levels between sickle cell disease patient in steady state (A) and non-sickle cell controls (p = 0.005 and 0.000) respectively as well as between sickle cell disease patient in VOC (B) and non-sickle cell controls (p = 0.000). There were no statistically significant differences in the SOD and GPX levels of sickle disease subjects based on age, gender, maternal level of educational attainment, occupational group and income (p = 0.629 and 0.476; p = 0.382 and 0.417; p = 0.450 and 0.314 and p = 0.397 and 0.762 and p = 0.553 and 0.929) respectively. There were no statistically significant differences in the SOD and GPX levels of sickle disease subjects of Hausa/Fulani extraction versus Yoruba (p = 0.714 and 0.856), between Hausa/Fulani extraction versus Igbo (0.917 and 0.486) and between Yoruba extraction versus Igbo (p = 0.740 and 0.965) respectively. This study confirms that SCD children have lower values of antioxidant enzymes compared to controls. SOD and GPX levels in sickle cell disease patient in steady state and vaso-occlusive crisis are significantly lower compared that of non-sickle cell controls. Patients with SCD may benefit from substances with antioxidant properties which can potentially reduce the complications associated with the disease.
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Introduction Sickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking. Methods A retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ ² or Fisher’s exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ ² or Fisher’s exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay. Results There were 16 333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95% CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×10 ⁹ /L) 358.8 (SD=200.9) while median value for white cell count (×10 ⁹ /L) was 23.5 (IQR: 18.0–31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5 days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication. Conclusion Anaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.
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ABSTRACT Introduction Sickle cell disease (SCD) remains a major cause of childhood mortality and morbidity in Malawi. However, literature to comprehensively describe the disease in the paediatric population is lacking. Methods A retrospective review of clinical files of children with SCD was conducted. Descriptive statistics were performed to summarise the data. χ2 or Fisher’s exact test was used to look for significant associations between predictor variables and outcome variables (case fatality and length of hospital stay). Predictor variables that were significantly associated with outcome variables (p≤0.05) in a χ2 or Fisher’s exact test were carried forward for analysis in a binary logistic regression. A multivariable binary logistic regression was used to identify covariates that independently predicted length of hospital stay. Results There were 16333 paediatric hospitalisations during the study period. Of these, 512 were patients with SCD representing 3.1% (95% CI: 2.9%- 3.4%). Sixty-eight of the 512 children (13.3%; 95%CI: 10.5% - 16.5%) were newly diagnosed cases. Of these, only 13.2% (95% CI: 6.2% - 23.6%) were diagnosed in infancy. Anaemia (94.1%), sepsis (79.5%) and painful crisis (54.3%) were the most recorded clinical features. The mean values of haematological parameters were as follows: haemoglobin (g/dL) 6.4 (SD=1.9), platelets (×109 /L) 358.8 (SD=200.9) while median value for white cell count (×109 /L) was 23.5 (IQR: 18.0–31.2). Case fatality was 1.4% (95% CI: 0.6% - 2.8%)and 15.2% (95% CI: 12.2% -18.6%) of the children had a prolonged hospital stay (>5days). Patients with painful crisis were 1.7 (95% CI: 1.02 - 2.86) times more likely to have prolonged hospital stay than those without the complication. Conclusion Anaemia, sepsis and painful crisis were the most common clinical features paediatric patients with SCD presented with. Patients with painful crisis were more likely to have prolonged hospital stay. Delayed diagnosis of SCD is a problem that needs immediate attention in this setting. Although somewhat encouraging, the relatively low in-hospital mortality among SCD children may under-report the true mortality from the disease considering community deaths and deaths occurring before SCD diagnosis is made.
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Background: Characterization of hematological parameters in patients with sickle cell anemia can direct treatment goals toward strategies reducing morbidity and mortality rate.Objective: To evaluate the hematological parameters in a subset of Iraqi patients with sickle cell anemia in relation to age and gender. Patients and Methods: This cross-sectional study was included 30 patients with sickle cell anemia who were known to be at a steady clinical state with no vaso-occlusive crises. About 5 ml of venous blood was collected in EDTA tube from each participant. The complete blood count was conducted according to standard protocol. Results: The mean age was 7.93 ±2.43 years and the age range was 4 to 12 years. The study included 17 males and 13 females. 29 (96.7 %) were anemic and there was no significant difference in mean hemoglobin levels between males and females (p = 0.838). Males demonstrated higher frequency of low mean cell volume (MCV) and mean cell hemoglobin (MCH) (64.7% and 58.8%, respectively) than females (7.7% and 7.7%, respectively) with highly significant differences. Conclusion: Some hematological parameters in patients with sickle disease are affected by gender but show no significant correlation to age. Keywords: Hematological characteristics, Sickle cell anemia, Iraq
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We have studied serum immunoreactive erythropoietin (SIE) levels in 28 patients with sickle cell anemia (SCA) without renal insufficiency and in 17 patients with nonhemoglobinopathy anemias of comparable severity using a sensitive radioimmunoassay procedure. An exponential relationship between SIE level and degree of anemia was noted in all patients. However, in nonhemoglobinopathy anemia, a sharp rise in the SIE level occurred as hemoglobin (Hb) levels fell below about 12 g/dL, whereas in sickle cell patients the increase was not marked until hemoglobin fell to about 9 g/dL. The response was more blunted in older SCA patients than in younger ones. A linear regression model relating SIE level to Hb level, presence/absence of SCA, and age explained 63% of the variation in SIE. We conclude that the serum erythropoietin levels in SCA increased at a lower hemoglobin concentration and are of a lower magnitude than that of the other anemias.
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Sickle cell disease is a genetic haemoglobinopathy with consequent haemolysis and anaemia. It is of interest to study its effect on red cell indices beside haemoglobin concentration. The objective of the study is to determine the values of red cell indices in preschool-age children with sickle cell anaemia. we conducted a cross-sectional study including 97 children with sickle cell anaemia aged six months to five years and 97 age-and sex-matched healthy controls with haemoglobin genotype AA (Hb AA). The red cell indices such as packed cell volume, haemoglobin concentration, mean corpuscular volume, red blood cell count, mean corpuscular haemoglobin and mean corpuscular haemoglobin concentration were investigated, using an auto analyzer. The mean PCV, haemoglobin concentration and red blood cell count were significantly higher in HbAA controls (p = 0.000 in each case). The mean MCV was higher among HbSS subjects but it was only among females and when the result was analyzed irrespective of gender that the difference was statistically significant (p < 0.05). Children with sickle cell anaemia in steady state have lower values of all red cell parameters and higher values of MCV, compared to haemoglobin phenotype AA controls.
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Introduction Sickle cell disease (SCD) is the most common inherited disorder of haemoglobin worldwide. This study evaluated the chromatographic patterns and red blood cell indices of sickle cell patients to determine the co-inheritance of other haemoglobin(Hb) variants and β-thalassaemia trait. Methods Red cell indices, blood film, sickle solubility test, Hb electrophoresis using alkaline cellulose acetate membrane, and chromatographic patterns using Bio Rad HPLC Variant II were evaluated for 180 subjects. Results Based on low MCV <76fL and MCH<25 pg, in the presence of elevated A2 >4.0% on HPLC and Hb variants eluting outside the S and C windows, at least four haemoglobin phenotypes (SS: 87.7%; SC: 1.1%; SD Punjab: 0.6%; Sβ-thalassemia: 10.6%) were identified. Mean Hb F% was 8.1±5.1 (median 7.65) for Hb SS and 6.03±5.2 (median 3.9) for Hb Sβ-thalassemia trait. Majority of Hb SS (69.1%) had Hb F% less than 10 while 27.6% had 10-19.9 and 3.2% had ≥ 20. Mean Hb F% was higher in female Hb SS (9.55±5.09; mean age 7.4±3.8 years) than the males (7.63±4.80; mean age 6.9±3.8 years) (P=0.02). A borderline significant negative correlation between age and Hb F levels among Hb SS subjects (r= -0.169 P=0.038) was also observed. Conclusion Our data suggests that α and β- thalassaemia traits, and other haemoglobin variants co-exist frequently with SCD in our population
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Background: In Africa, SCD is the most common inherited hematological disease with high mortality rate at age one to five years. This disease was discovered early in Sudan, the peak occurrence of SCD is among the population from the Western Sudan. The objective of this study was to determine the haematological parameters changes in Sudanese patients with SCD . Methods: This study was conducted in Omdurman between August 2011 - Aril 2012, in hundred patients with homozygous SCD of age between 6 month –17 years. Questionnaires were used to collect demographic and clinical data. About 5 ml of venous anticoagulated blood were collected for complete blood count. Results A total of hundred cases were enrolled. More than 97% belong to tribes from Western Sudan. The Hb , RBCs count and Hct level were low in all patients. The normal MCH and MCHC was observed except in age group below two years. The TWBCs were significantly increased in all cases. Conclusions: This study provides haematological reference ranges for homozygous Sudanese patients with SCD. As well is known, the results of this study showed lower values of Hb, RBCs count and Hct among patients. The remarkable result in this study was the significant increase in leucocytes count.
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CONTEXT AND OBJECTIVES Sickle cell disease (SCD) is the most common genetic disorder among people of African descent, affecting approximately 3,500 newborns each year in Brazil. Hydroxyurea (HU) is the only effective drug to treating patients with SCD, thereby reducing morbidity and mortality. The objective was to analyze the effects of HU on SCD patients at our institution. DESIGN AND SETTING Retrospective study conducted at a sickle cell centre in Ribeirão Preto, São Paulo, Brazil. METHODS We analyzed clinical and laboratory data on 37 patients. The hematological parameters and clinical events that occurred during the year before and the first year of treatment with HU were analyzed. The mean dose of HU was 24.5 ± 5.5 mg/kg/day. RESULTS There were rises in three parameters: hemoglobin (8.3 g/dl to 9.0 g/dl, P = 0.0003), fetal hemoglobin (HbF) (2.6% to 19.8%, P < 0.0001) and mean cell volume MCV (89 to 105 fl, P = 0.001); and reductions in the numbers of leukocytes (10,050/µl to 5,700/µl, P < 0.0001), neutrophils (6,200/µl to 3,400/µl, P = 0.001), platelets (459,000/µl to 373,000/µl, P = 0.0002), painful crises (1.86 to 0.81, P = 0.0014), acute chest syndromes (0.35 to 0.08, P = 0.0045), infections (1.03 to 0.5, P = 0.047), hospitalizations (1.63 to 0.53, P = 0.0013) and transfusions (1.23 to 0.1, P = 0.0051). CONCLUSION The patients presented clinical and hematological improvements, with an increase in HbF and a reduction in the infection rate, which had not been addressed in most previous studies.
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Sickle cell anemia (SCA) has multisystemic manifestations and is associated with severe morbidity and high mortality. It commonly affects growth leading to wasting and stunting. This study aimed to determine the influence of socioeconomic status on the nutritional status using anthropometric measurements and steady-state hemoglobin, of children with homozygous SCA, aged 1 year to 10 years in steady state at the Lagos University Teaching Hospital. This is a cross-sectional study involving 100 children with SCA and 100 age-, sex-, and social class-matched controls that fulfilled the inclusion criteria. Social class was assessed using educational attainment and occupation of parents. Hemoglobin concentration was determined using the oxy-hemoglobin method. This study demonstrated a significantly lower mean weight and weight-for-height in the SCA patients than those of controls (P < 0.001). By contrast, this study did not demonstrate any statistical significant difference in the mean height and mean body mass index of SCA patients and controls (P = 0.06) and (P = 0.12), respectively. The mean weight, height, and body mass indices of the subjects and controls were consistently below those of the NCHS standards. The magnitude of the difference from the NCHS standard was also more pronounced in the subjects, increased with advancing age and affected male subjects more than females. Progressive declines in the anthropometric attainment and hemoglobin concentration were observed from social class 1 to 4; this was statistically significant in controls (P = 0.00) but not in subjects (P > 0.1). However, SCA patients had significantly lower values than controls in each of the social classes. Poor socioeconomic status has an adverse effect on the nutritional status and hemoglobin of SCA patients.
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Background:Sickle cell anaemia (SCA) is a major cause of morbidity and mortality in Africa where there is no readily available effective treatment. This study was designed to determine the haematological values that can be used in monitoring the status and management of SCA patients. Method: A prospective study of 200 patients (81.3%) in steady state, 46 patients (18.7%) during vaso-occlusive crisis (VOC) and 84 control subjects seen between August 2001 and July 2002 in 3 centers in Benin City, Nigeria had their blood samples analyzed within two hours of collection. Automated Coulter Counter was used to determine the complete blood counts while the foetal haemoglobin (HbF) was estimated by the modified Betke method and haemoglobin A2 by HbS-free microcolumn chromatography. Results: The mean cell volume (MCV), mean cell haemoglobin (MCH) and mean cell haemoglobin concentration (MCHC) in steady state were 79.38fl ± 22.41, 28.31pg ± 3.58 and 32.56g/dl ± 2.27 while in VOC they were 85.50fl ± 8.14, 28.79pg ± 2.78 and 33.76g/dl±3.44 respectively. The red cell distribution width (RDW), haemoglobin A2 and F in steady state were 23.76% ± 6.49, 4.52% ±1.16 and 2.17% ±1.81 while during VOC they were 21.62% ±5.11, 3.82%±1.27 and 2.05%±1.19 respectively. The neutrophil count (P<0.01), MCV (P<0.01) and MCHC (P<0.05) were significantly higher during VOC than steady state while the RDW (P<0.05) and haemoglobin A2 were significantly higher in steady state than during VOC. Conclusion:Parameters, which are not usually reported in previous studies, have been shown in steady state and VOC.
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To determine the clinical, haematological, biochemical and rheological changes that occur in the asymptomatic steady state of sickle cell anaemia. Patient self-assessment visual analogue scores (for wellbeing and tiredness), the blood concentration of acute phase proteins (C-reactive protein, orosomucoid, and fibrinogen), and blood rheology (percentage of dense cells and the number of sickled cells that occluded pores 5 microns in diameter) were studied longitudinally on 10 occasions in each of 20 outpatients with sickle cell anaemia. Patients in the steady state showed fluctuation in visual analogue scores, in concentration of acute phase proteins, and in rheological parameters consistent with minor episodes of tissue injury. Significantly more variation in acute phase proteins occurred in the steady state of 14 of the 20 patients who developed one or more vaso-occlusive crises during the 16 month study period. Rheological fluctuation in the steady state simulated rheological change during crisis, namely a transient rise and then fall in the number of dense and poorly filterable cells. The term "steady state" is a misnomer, being characterised by biochemical and rheological fluctuation consistent with minor episodes of microvascular occlusion that are insufficient to cause the overt tissue infarction of painful crisis.
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We have studied serum immunoreactive erythropoietin (SIE) levels in 28 patients with sickle cell anemia (SCA) without renal insufficiency and in 17 patients with nonhemoglobinopathy anemias of comparable severity using a sensitive radioimmunoassay procedure. An exponential relationship between SIE level and degree of anemia was noted in all patients. However, in nonhemoglobinopathy anemia, a sharp rise in the SIE level occurred as hemoglobin (Hb) levels fell below about 12 g/dL, whereas in sickle cell patients the increase was not marked until hemoglobin fell to about 9 g/dL. The response was more blunted in older SCA patients than in younger ones. A linear regression model relating SIE level to Hb level, presence/absence of SCA, and age explained 63% of the variation in SIE. We conclude that the serum erythropoietin levels in SCA increased at a lower hemoglobin concentration and are of a lower magnitude than that of the other anemias.
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To understand the contribution to the pathophysiology of sickle cell anemia of the different erythrocyte density types present in the blood of these patients, we have studied the viscosimetric and hemodynamic characteristics of four major classes of hemoglobin SS erythrocytes. We have isolated reticulocytes, discocytes, dense discocytes, and irreversibly sickled cells (fractions I-IV) on Percoll-Renografin density gradients. Bulk viscosity was studied in a coneplate viscosimeter and the hemodynamic studies were performed on the isolated, artificially perfused mesoappendix vasculature of the rat (Baez preparation). Bulk viscosity measurements at shear rates of 230 S-1 demonstrate that when the cells are oxygenated, fraction I (reticulocyte rich) has a higher viscosity than expected from its low intracellular hemoglobin concentration. The rest of the fractions exhibit moderate increases in bulk viscosity pari-passu with the corresponding increases in density (mean corpuscular hemoglobin concentration). When deoxygenated, all cell fractions nearly doubled their bulk viscosity and the deoxy-oxy differences remained constant. The Baez preparation renders a different picture: oxygenated fractions behave as predicted by the viscosimetric data, but, when deoxygenated, cell fractions exhibit dramatically increased peripheral resistance and the deoxy-oxy difference are directly proportional to cell density, thus, the largest increases were observed for fractions III and IV. The differences between the rheological and the hemodynamic measurements are most probably due to the different sensitivity of the two methods to the extent of intracellular polymerization. These results also demonstrate that the hitherto unrecognized fraction III cells (very dense discocytes that change shape very little on deoxygenation) are as detrimental to the microcirculation as the irreversibly sickled cell-rich fraction IV. They may, however, induce obstruction by a different mechanism. As the extent to which these fractions are populated by erythrocytes varies considerably from patient to patient, the distribution function of cell densities in each sickle cell anemia patient might have consequences for the type of pathophysiological events occurring in their microcirculation.
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Cerebrovascular accident (CVA) is a major complication of sickle cell disease. The incidence and mortality of and risk factors for CVA in sickle cell disease patients in the United States have been reported only in small patient samples. The Cooperative Study of Sickle Cell Disease collected clinical data on 4,082 sickle cell disease patients enrolled from 1978 to 1988. Patients were followed for an average of 5.2 +/- 2.0 years. Age-specific prevalence and incidence rates of CVA in patients with the common genotypes of sickle cell disease were determined, and the effects of hematologic and clinical events on the risk of CVA were analyzed. The highest rates of prevalence of CVA (4.01%) and incidence (0.61 per 100 patient-years) were in sickle cell anemia (SS) patients, but CVA occurred in all common genotypes. The incidence of infarctive CVA was lowest in SS patients 20 to 29 years of age and higher in children and older patients. Conversely, the incidence of hemorrhagic stroke in SS patients was highest among patients aged 20 to 29 years. Across all ages the mortality rate was 26% in the 2 weeks after hemorrhagic stroke. No deaths occurred after infarctive stroke. Risk factors for infarctive stroke included prior transient ischemic attack, low steady-state hemoglobin concentration and rate of and recent episode of acute chest syndrome, and elevated systolic blood pressure. Hemorrhagic stroke was associated with low steady-state hemoglobin and high leukocyte count.
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This is the first report of a method to assess the significance of numerical changes in the platelet count based upon a result exceeding the normal intra-individual variation in platelet numbers. Serial platelet counts from 3,789 subjects were analysed to determine the intra-individual variation in platelet numbers. A platelet count difference of 98 x 10(9)/L in males was found to represent a change that would occur by chance in less than 1 in 1,000 platelet count determinations. Tables to determine the significance of platelet number variations, given N previous observations, are provided at two probability levels. The repeatability of the platelet count was calculated as 0.871 (males) and 0.849 (females) indicating that the heritability of platelet count is high and that the platelet count is predominantly genetically determined. A seasonal variation in platelet count was found with a 'winter' versus 'summer' difference of 5.10 X 10(9)/L (males) and 5.82 x 10(9)/L (females).
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Hydroxyurea improves hematologic parameters for children with sickle cell disease (SCD), but its long-term efficacy at maximum tolerated dose (MTD) has not been determined. Between 1995 and 2002, hydroxyurea therapy was initiated for 122 pediatric patients with SCD including 106 with homozygous sickle cell anemia (HbSS), 7 with sickle hemoglobin C (HbSC), 7 with sickle/beta-thalassemia (HbS/ beta-thalassemia [6 HbS/beta0, 1 HbS/beta+]), and 2 with sickle hemoglobin OArab (HbS/OArab). Median age at initiation of therapy was 11.1 years. Hydroxyurea was escalated to MTD, with an average dose of 25.4 +/- 5.4 mg/kg per day; the average duration of hydroxyurea therapy has been 45 +/- 24 months (range, 6-101 months). Hydroxyurea was discontinued for 15 (12%) children with poor compliance. Mild transient neutropenia occurred, but no hepatic or renal toxicity was noted. Hydroxyurea therapy led to significant increases in hemoglobin level, mean corpuscular volume, and fetal hemoglobin (HbF) level, whereas significant decreases occurred in reticulocyte, white blood cell, and platelet counts and serum bilirubin levels. Children with variant SCD genotypes also had hematologic responses to hydroxyurea. HbF induction has been sustained for up to 8 years without adverse effects on growth or increased numbers of acquired DNA mutations. Long-term hydroxyurea therapy at MTD is well tolerated by pediatric patients with SCD and has sustained hematologic efficacy with apparent long-term safety.
Article
Eight adult patients with sickle cell anemia were followed over a 6-mo period. During this time all patients had elevated plateletcounts, 1.7- fold (mean, 438,398 +/- 86,223), and megathrombocyte numbers, 2.3-fold (mean, 79,535 +/- 38,907), during asymptomatic periods. These data are interpreted as suggesting that the elevated platelets and megathrombocytes in asymptomatic sickle cell patients result from lack of splenic sequestration. During three crises in two patients, both megathrombocyte number and platelet count fell significantly. It is speculated that platelets may be associated with the pathology in this disease. Platelet function studies performed during crises, therefore, must take into account the number of megathrombocytes.
Article
We have collected steady-state laboratory data for over 2600 patients, age 2 years and over, with sickle cell anemia (HbSS), HbSC disease, and HbS-beta+-thalassemia. The packed cell volume (PCV) is lower in males than in females until 17 or 18 years of age in HbSS and ages 13 to 15 in HbSC, but then becomes consistently higher in males. After age 40, the PCV falls in HbSS. The steady-state leukocyte count in HbSS is higher than that in normals, blunting the utility of this measurement in the assessment of infection. In HbSC and HbS-beta+-thalassemia, the leukocyte counts are more often within the range of normal. Platelet counts in HbSS are often found to be above normal and show a downward trend with age. There is a progressive rise in creatinine with age. In HbSS, this rise begins at age 14 and may be accounted for by the increased muscle mass that occurs with puberty. The further deterioration of renal function in patients over 20 may be a result of the known adverse effects of sickle cell disease upon the kidney. Our data provide a basis to compare perturbations caused by intercurrent complications and new therapies, as well as to contrast with similar information from other populations of patients with sickle cell disease.
Article
Introduction: Sickle cell disease (SCD) has a wide range of clinical presentation. We evaluated hematological parameters, which are widely evaluable and assessable, as indices of clinical outcome in SCD. These parameters, although largely established as indices of clinical outcome in other SCD populations, have not been widely evaluated in African patients. Materials and methods: One hundred and thirty six consecutive stable SCD patients who presented in the sickle cell clinic of a teaching hospital were studied retrospectively using a questionnaire. Hematological parameters of full blood count (FBC) for each patient were obtained using a cell counter. FBC parameters such as White blood cell count (WBC) were then statistically correlated with complications such as ankle ulcers, osteomylitis and others. A Chi-square text was used to compare frequencies and generate P values. Results: The presence of sickle cell complications was significantly associated with raised white blood cell count (WBC) above 11 × 10(9)/l (P = 0.03).The WBC of the patients increased with increasing numbers of complications (P = 0.07). Mean packed cell volume (PCV) and WBC tended toward the reference range for age and sex (in apparently normal individuals) as the age at diagnosis of SCD increased. This trend was significant for PCV (P = 0.01). Conclusion: Our data provide additional support that widely evaluable and assessable hematological parameters such as PCV and WBC can be used as indices to predict SCD outcome in African patients. This is likely to impart positively on individualized therapy.
Chapter
IntroductionGeographic distribution of sickle mutationPathophysiologyClinical manifestationsVariant sickle cell syndromesDiagnosisTherapySelected bibliography
Article
We studied thrombopoietin (TPO, Mpl ligand) values using a sensitive ELISA in 254 serum samples obtained from disease-free children and adult volunteers. TPO was detected in all samples, and its values ranged widely from 0.25 to 9.18 fmol/ml. When analysed by dividing the subjects into 11 age groups, the mean TPO levels from birth to 1 month of age were increased (3.73–5.92 fmol/ml). The highest values were found 2 d after birth; TPO levels then gradually decreased to adult levels (0.83 fmol/ml). The relationship between TPO values and platelet counts was not significant in all subjects (r = 0.27) or in children alone (r = 0.12). In children > 1 month of age a 95% reference interval for serum TPO values was determined from 0.58 to 3.27 fmol/ml. A significant correlation was found between TPO values in serum and plasma; serum TPO values = −0.257 + 4.039 × plasma TPO values (r = 0.951, P < 0.001, n = 22). This study is the first to report age-dependent changes in blood TPO levels throughout child development. Serum TPO values were significantly high up to 1 month of age and were correlated with plasma TPO levels.
Article
Eight adult patients with sickle cell anemia were followed over a 6-mo period. During this time all patients had elevated plateletcounts, 1.7-fold (mean, 438,398 +/- 86,223), and megathrombocyte numbers, 2.3-fold (mean, 79,535 +/- 38,907), during asymptomatic periods. These data are interpreted as suggesting that the elevated platelets and megathrombocytes in asymptomatic sickle cell patients result from lack of splenic sequestration. During three crises in two patients, both megathrombocyte number and platelet count fell significantly. It is speculated that platelets may be associated with the pathology in this disease. Platelet function studies performed during crises, therefore, must take into account the number of megathrombocytes.
Article
Acute episodes of pain are the principal symptom of sickle cell disease, but little is known about the epidemiologic features of these episodes or risk factors for them, nor is it known whether patients with high rates of such episodes die prematurely. We prospectively studied the natural history of sickle cell disease in 3578 patients ranging from newborns to persons up to 66 years old who were followed at clinical centers across the United States. There were 12,290 episodes of pain in 18,356 patient-years. The average rate was 0.8 episode per patient-year in sickle cell anemia, 1.0 episode per patient-year in sickle beta 0-thalassemia, and 0.4 episode per patient-year in hemoglobin SC disease and sickle beta(+)-thalassemia. The rate varied widely within each of these four groups--e.g., 39 percent of patients with sickle cell anemia had no episodes of pain, and 1 percent had more than six episodes per year. The 5.2 percent of patients with 3 to 10 episodes per year had 32.9 percent of all episodes. Among patients with sickle cell anemia who were more than 20 years old, those with high rates of pain episodes tended to die earlier than those with low rates. High rates were associated with a high hematocrit and low fetal hemoglobin levels. alpha-Thalassemia had no effect on pain apart from its association with an increased hematocrit. The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.
Article
A study of the natural history of sickle hemoglobinopathies was begun in March 1979. By August 1987, a total of 2824 patients less than 20 years of age were enrolled. There have been 14,670 person-years of follow-up. Seventy-three deaths have occurred. Most of the deaths were in patients with hemoglobin SS. The peak incidence of death was between 1 and 3 years of age, and the major cause in these young patients was infection. Cerebrovascular accidents and traumatic events exceeded infections as a cause of death in patients greater than 10 years of age. There was limited success in identifying risk factors for death. Comparison of this study's overall mortality of 2.6% (0.5 deaths per 100 person-years) with previous reports indicates improvement of survival in US patients less than 20 years of age with sickle hemoglobinopathies. This improvement is most likely due to parental education and counseling about the illness and the early institution of antibiotics in suspected infections.
Article
The hospital records of 62 Zambian children with sickle cell anaemia (SCA) who died during a 3 year period (January 1987 to December 1989) at the Paediatric Wing of the University Teaching Hospital, Lusaka, Zambia, were reviewed retrospectively. The SCA patients accounted for 2.92 percent of the total admissions and the average case fatality was 6.61 percent of the total SCA admissions. The case fatality rate has reduced considerably as compared to the one observed in 1970 in Zambia, although the major causes of death remain the same. The maximum mortality was noted in the age group of one to five years (54.84%). The common causes of death were infections (29.54%), vasoocclusive crises (22.72%) and splenic sequestration crises (20.45%). The problems of sub-Saharan Africa, like malaria, malnutrition and now the HIV infection also adde to the mortality (15.90%).
Article
Physiological platelet synthesis is thought to require the humoral activities of meg-CSF and thrombopoietin, which respectively promote proliferation and maturation of megakaryocytic cells. A meg-CSF/thrombopoietin-like protein that is present in plasma of irradiated pigs has been purified and cloned. This protein binds to and activates the c-mpl protein, a member of the cytokine receptor superfamily. The isolated Mpl ligand shares homology with erythropoietin and stimulates both megakaryocytopoiesis and thrombopoiesis.
Article
Sickle cell disease is a serious public health problem in Gabon with a relatively high mortality rate. Charts of 23 children (nine boys, 14 girls) who died of complications from sickle cell anemia in the department of pediatrics of Owendo Pediatric Hospital (Libreville, Gabon), from January 1, 1990 through December 31, 1992, were analysed retrospectively. Approximately two-thirds of the children (60.9%) were under 5 years of age. The great majority of patients were from low socio-economic standard families. Of 319 deaths observed during the study period, 23 were due to sickle cell disease-associated complications, for an overall mortality rate of 7.2% and a related mortality of 3.6%. Commonest causes of deaths were severe anemia (11 cases, i.e., 47.8%), which affected predominantly the younger patients between 6 months and 5 years (eight cases), infections (30.4%) and blood transfusion complications (21.7%). To decrease these mortality rates, appropriate health supervision and well-designed preventive strategies are needed.
Article
The relationship between each of haemoglobin concentration (Hb), red cell count (RCC), white cell count (WBC), platelet count (PLT), mean corpuscular volume (MCV), red cell width difference (RWD), and average number of acute admissions per year (AVEADM), were determined in a cross-sectional study of 128 Afro-Caribbean sickle cell anaemia patients attending the Sickle Cell Disease Clinic of Central Middlesex Hospital in London for a mean of 7.6 patient-years. The diagnosis of sickle cell anaemia was made by both haematological and DNA analyses. The haematological parameters were determined using Coulter S automated counter during the steady state periods while the AVEADM was computed from all admissions for painful crisis, acute anaemia and acute chest syndrome, priapism, and acute stroke. Haemoglobin F level was determined by HPLC. Analysis was done in the paediatric and adult patients separately. There were no significant correlations between any of the parameters and AVEADM in the paediatric group. In adult patients, there was significant positive correlation (P < 0.05) between WBC and AVEADM. WBC has a negative correlation with Hb concentration and HbF level. WBC 10 x 10(9)/L and above is associated with Hb and HbF level below the mean for the group. WBC is lower but not significantly, in patients with single alpha-gene deletion than in those without deletion (P = 0.06). This study suggests that higher WBC count may suggest possible increased hospital admission, lower Hb concentration, and lower HbF level, in adult patients, and that, as a single parameter it can be of value in the assessment of patients with sickle cell anaemia. Possible mechanisms for these findings are also suggested.
Article
Sickle cell disease (SCD) is characterized by a point mutation that replaces adenine with thymidine in the sixth codon of the beta-globin gene, a unique morphological abnormality of red blood cells, vaso-occlusion with ischaemic tissue injury, and susceptibility to infections. Vascular lumen obstruction in SCD results from interaction of erythrocytes, leukocytes, platelets, plasma proteins, and the vessel wall. The disease phenotype is a product of various genes and environmental factors acting in concert with the protein lesion underlying the red cell anomaly. The severity of SCD increases with leukocyte count. The biological basis and therapeutic implications of this relationship are discussed. Leukocytes contribute to SCD by adhering to blood vessel walls and obstructing the lumen, aggregating with other blood cells with more effective blockage of the lumen, stimulating the vascular endothelium to increase its expression of ligands for adhesion molecules on blood cells, and causing tissue damage and inflammatory reaction which predispose to vaso-occlusion. Patients with impaired ability of leukocytes to kill microbes are more prone to infections; which precipitate sickle cell crisis. Reduction of leukocyte count ameliorates SCD. Similarly, targeted blockade or reduced synthesis of specific leukocyte adhesion molecules and their ligands might confer clinical benefit in SCD.
Article
To determine the changes in serum erythropoietin with age in patients with and without anemia and to assess the importance of certain comorbidities on changes in erythropoietin level and the development of anemia. Clinical history, hematological parameters, and serum erythropoietin levels were examined at 1- to 2-year intervals for 8 to 30 years. Baltimore Longitudinal Study on Aging (BLSA), National Institute on Aging. One hundred forty-three BLSA participants. Complete blood count and serum chemistries were performed at the time of each visit, and archived serum samples were used for erythropoietin level. Although all subjects were healthy and without anemia at the time of initial evaluation, some developed chronic illness-most notably hypertension and diabetes mellitus. Erythropoietin levels rose significantly for the group as a whole, and the slope of the rise was found to be greater for those who did not have associated diabetes mellitus or hypertension. During the subsequent years, subjects who developed anemia but did not have hypertension or diabetes mellitus had the greatest slope in erythropoietin rise over time, whereas those with hypertension or diabetes mellitus and anemia had the lowest erythropoietin slope. The increase in serum erythropoietin with aging may be compensation for subclinical blood loss, increased red blood cell turnover, or increased erythropoietin resistance of red cell precursors. It is suspected that, with very advanced age, or in those with compromised renal function (e.g., diabetes mellitus or hypertension), the compensatory mechanism becomes inadequate and anemia results.
Article
This article discusses the importance of leukocyte adhesion in sickle cell disease, and how this could be modulated for clinical benefit. Recurrent inflammation and vasculopathy occur in sickle cell disease. As a result, leukocytes and vascular endothelial cells are activated and increase their expression of adhesion molecules. Adhesion of leukocytes to other blood cells and endothelium contributes to vaso-occlusion in sickle cell disease. High-level expression of adhesion molecules by leukocytes is associated with clinically severe disease. Pancellular membrane lipid abnormalities, including reduced proportions of omega-3 fatty acids, occur in sickle cell disease. These lipid abnormalities are more severe in patients with disease complications and in those with a greater degree of anaemia. Since lipid constitution of cell membranes affects surface expression of adhesion molecules, the above findings could account for earlier observations that omega-3 fatty acids reduce P-selectin expression and reduce the frequency of sickle cell crisis. By inhibition of nuclear factor kappaB, glucocorticoids reduce activation of vascular endothelial cells, their expression of ligands for leukocyte adhesion molecules, and vaso-occlusion. Monoclonal antibodies to vascular endothelial intercellular adhesion molecule-1 inhibited hypoxia-induced vaso-occlusion in transgenic sickle mice. Although hydroxyurea and glucocorticoids reduce adhesion molecule expression by leukocytes and vascular endothelial cells, cytotoxicity and systemic side effects dampen enthusiasm for their use in sickle cell disease. Omega-3 fatty acids have shown promising efficacy and safety in pilot studies. A large clinical trial of these naturally occurring substances is required.
Sickle cell disease. In: Postgraduate Haematology
  • L Ashutosh
  • Pv Elliot
Ashutosh L, Elliot PV. Sickle cell disease. In: Postgraduate Haematology. 5 th Ed.
  • A V Hoffbrand
  • D Catovsky
  • Egd Tuddenham
Hoffbrand AV, Catovsky D, Tuddenham EGD (Eds), Blackwell. 2005;104-118.