Hematology Research and Reviews

Publications in this journal

• Article: Lipid profile of regular blood donors.

  Ei Uche, A Adediran, Od Damulak, Ta Adeyemo, Aa Akinbami, As Akanmu
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  ABSTRACT: A few reports have linked regular blood donation to the lowering of parameters of lipid profile. Estimating the lipid profile is an accepted method of assessing an individual's risk for coronary heart disease, particularly if there is evidence of lipid peroxidation. Regular blood donation may lower iron stores, and this in turn lowers lipid peroxidation. This study was carried out to determine the effect of blood donation on lipid profile. Eighty-two participants consented to participate and were enrolled into the study, 52 of whom were regular blood donors (study group) and 30 were non-donors (control group). Venous blood (10 mL) was drawn from each subject into new plain screw-capped disposable plastic tubes. This was allowed to clot and the serum was used to determine total cholesterol, triglycerides, low-density lipoprotein, and high-density lipoprotein. The mean total cholesterol (4.66 ± 0.86 mmol/L), triglycerides (1.22 ± 0.64 mmol/L), and low-density lipoprotein (2.32 ± 0.73 mmol/L) were significantly lower in the regular blood donors than the control group (5.61 ± 1.26 mmol/L, 1.77 ± 2.9 mmol/L, and 3.06 ± 0.89 mmol/L, respectively; P < 0.05 in all cases). Also, while 42% of the study group had a low/high-density lipoprotein ratio of at least three, about 57% of the control group had a ratio of at least three (P = 0.21). Regular blood donation may be protective against cardiovascular disease as reflected by significantly lower mean total cholesterol and low-density lipoprotein levels in regular blood donors than in non-donors.
  Hematology Research and Reviews 01/2013; 4:39-42.
• Article: Beta-amyloidolysis and glutathione in Alzheimer's disease.

  J Lasierra-Cirujeda, P Coronel, Mj Aza, M Gimeno
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  ABSTRACT: In this review, we hypothesized the importance of the interaction between the brain glutathione (GSH) system, the proteolytic tissue plasminogen activator (t-PA)/plasminogen/ plasmin system, regulated by plasminogen activator inhibitor (PAI-1), and neuroserpin in the pathogenesis of Alzheimer's disease. The histopathological characteristic hallmark that gives personality to the diagnosis of Alzheimer's disease is the accumulation of neurofibroid tangles located intracellularly in the brain, such as the protein tau and extracellular senile plaques made primarily of amyloidal substance. These formations of complex etiology are intimately related to GSH, brain protective antioxidants, and the proteolytic system, in which t-PA plays a key role. There is scientific evidence that suggests a relationship between aging, a number of neurodegenerative disorders, and the excessive production of reactive oxygen species and accompanying decreased brain proteolysis. The plasminogen system in the brain is an essential proteolytic mechanism that effectively degrades amyloid peptides ("beta-amyloidolysis") through action of the plasmin, and this physiologic process may be considered to be a means of prevention of neurodegenerative disorders. In parallel to the decrease in GSH levels seen in aging, there is also a decrease in plasmin brain activity and a progressive decrease of t-PA activity, caused by a decrease in the expression of the t-PA together with an increase of the PAI-1 levels, which rise to an increment in the production of amyloid peptides and a lesser clearance of them. Better knowledge of the GSH mechanism and cerebral proteolysis will allow us to hypothesize about therapeutic practices.
  Hematology Research and Reviews 01/2013; 4:31-8.
• Article: Serum ferritin levels in adults with sickle cell disease in Lagos, Nigeria.

  Akinsegun A Akinbami, Adedoyin O Dosunmu, Adewumi A Adediran, Olajumoke O Oshinaike, Vincent O Osunkalu, Sarah O Ajibola, Olanrewaju M Arogundade
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  ABSTRACT: Serum ferritin is considered to be one of the most important tools in the measurement of iron balance in steady-state sickle cell disease. Increased gastrointestinal absorption of iron has been reported in sickle cell disease because of the associated chronic hemolysis, and it is also thought that repeated red cell transfusion consequent to chronic hemolysis and anemia causes excessive iron levels. The aim of this study was to determine overall and gender-specific mean ferritin levels in patients with steady-state sickle cell disease in order to establish the prevalence of iron deficiency and overload. This was a cross-sectional study in homozygous patients with sickle cell disease attending the sickle cell clinic at Lagos State University Teaching Hospital, Ikeja. A 5 mL blood sample was collected in plain bottles from consenting participants during steady-state periods. The serum was separated and analyzed for ferritin by enzyme-linked immunosorbent assay. Another 5 mL sample was collected for a full blood count, done on the same day of collection, to determine red blood cell indices, ie, mean cell volume, mean cell hemoglobin concentration, and mean corpuscular hemoglobin concentration. The Pearson Chi-square test was used for statistical analysis. The differences were considered to be statistically significant when P was <0.05. In total, 103 patients were recruited for this study and comprised 58 women (56.40%) and 45 men (43.70%). The overall mean ferritin concentration was 93.72 ± 92.24 ng/mL. The mean ferritin concentration in the women was 92.00 ± 88.07 ng/mL and in men was 96.41 ± 99.80 ng/mL. Only eight (7.76%) of the 103 patients had a serum ferritin level < 15 ng/mL, while two subjects (1.94%) had a serum a ferritin level > 300 ng/mL. Ninety-three subjects (90.29%) had serum ferritin within the normal reference range of 15-300 ng/mL. In this study, 90% of subjects with sickle cell disease had normal iron stores; serum ferritin was higher in men than in women, and iron deficiency was more common than overload in the disease.
  Hematology Research and Reviews 01/2013; 4:59-63.
• Article: New developments in the management of congenital Factor XIII deficiency.

  Zehra Fadoo, Quratulain Merchant, Karim Abdur Rehman
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  ABSTRACT: Congenital Factor XIII (FXIII) deficiency is a rare, inherited, autosomal recessive coagulation disorder. Most mutations of this condition are found in the A-subunit with almost half these being missense mutations. Globally, approximately one in three million people suffer from this deficiency. Factor XIII deficiency is associated with severe life threatening bleeding, intracranial hemorrhage, impaired wound healing, and recurrent pregnancy losses. FXIII is known to have a potential role in mediating inflammatory processes, insulin resistance, bone metabolism, neoplasia, and angiogenesis. The algorithm provided for FXIII diagnosis and classification will enable prompt identification and early intervention for controlling potential life threatening complications. Prophylactic replacement therapy using blood products containing FXIII such as fresh frozen plasma, cryoprecipitate, or using FXIII concentrate remains the mainstay for the management of FXIII deficiency. In most parts of the world, cryoprecipitate and plasma transfusions are the only treatments available. Management developments have revealed the effectiveness and safety of recombinant FXIII concentrate for prophylaxis and treatment. The aim of this review is to provide an overview of advancements made in the management of FXIII deficiency from the time it was first detected, highlighting novel developments made in recent years. Greater research is warranted in identifying novel approaches to manage FXIII deficiency in light of its underlying pathophysiology.
  Hematology Research and Reviews 01/2013; 4:65-73.
• Article: Public banking of umbilical cord blood or storage in a private bank: testing social and ethical policy in northeastern Italy.

  Sergio Parco, Fulvia Vascotto, Patrizia Visconti
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  ABSTRACT: In northeastern Italy, according to Italian legislation, authorized public facilities can accept the donation and preservation of cord blood stem cells (CB-SC). Attitudes and knowledge in pregnant women differs between the local and immigrant (non-European Union [EU]) population. In this study we assessed the choices that pregnant women have with respect to the public and private harvesting system and the main reasons driving their decisions. We examined the ethnic origin of the families and compared tests for syphilis screening and leukocyte (WBC) counts in the CB-SC bags that are required for validation of the collection. Out of a population of 3450 pregnant patients at the Institute for Maternal and Child Health of Trieste, northeast Italy, 772 women agreed to cord blood harvesting and the associated lab tests. Of these, 221 women (28.6%) were from immigrant families of non-EU countries. Their ethnic affiliation was recorded, and tests were performed for syphilis screening and for nucleated red blood cell (NRBC) interference with the WBC count in CB-SC bags to assess cellularity and to determine if storage was appropriate. Of the 772 pregnant women, 648 (84.0%) accessed the public collection system, which is free of charge, and 124 (15.0%) accessed the private fee-based system. One woman from the non-EU group opted for the private fee-based system. Of the 3450 pregnant women screened for syphilis at the Institute for Maternal and Child Health, the Treponema pallidum hemagglutination (TPHA) and Venereal Disease Research Laboratory (VDRL) tests were the main tests performed (66.0% of total cases) because many gynecologists in the public harvesting system apply the Italian regulations of the 1988 Decree, while the private system requires tests on syphilis and leaves the option to the lab physicians to select the best determination method. We found that the chemiluminescence method was more specific (97.0%) than the TPHA (83.0%) and nontreponemal rapid plasma reagin VDRL (75.0%) tests (P < 0.05, χ(2) test). The specificity link between the two automatic methods versus microscopes for WBC dosing and NRBC interference was r(2) = 0.08 (ADVIA 120) and r(2) = 0.94 (XE-2100). The public system does not include human T-cell lymphotropic virus testing; this is reserved for the population from endemic zones. In northeastern Italy current legislation prevents the establishment of private fee-based banks for storage of CB-SC. The cryopreservation, for future autologous personal or family use, is possible only by sending to foreign private banks, with a further fee of €300. These regulations confirm that Italian legislation tries to increase the anonymous allogenic donations and the number of CB-CS bags stored in the free-cost public system, that are available to anyone with therapeutic needs. Private banking is used almost exclusively by the wealthier local population. In the public system, many physicians continue to use older Italian laws regarding syphilis diagnosis, and NRBC interference on WBC count may have an impact on cord blood harvesting. Our findings suggest that in the EU there is no consensus policy on donor management. The value of storage for potential use within the family is useful only with collaboration between the public and the private systems.
  Hematology Research and Reviews 01/2013; 4:23-9.
• Article: Randomized double blind trial of ciprofloxacin prophylaxis during induction treatment in childhood acute lymphoblastic leukemia in the WK-ALL protocol in Indonesia.

  Pudjo H Widjajanto, Sumadiono Sumadiono, Jacqueline Cloos, Ignatius Purwanto, Sutaryo Sutaryo, Anjo Jp Veerman
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  ABSTRACT: Toxic death is a big problem in the treatment of childhood acute lymphoblastic leukemia (ALL), especially in low-income countries. Studies of ciprofloxacin as single agent prophylaxis vary widely in success rate. We conducted a double-blind, randomized study to test the effects of ciprofloxacin monotherapy as prophylaxis for sepsis and death in induction treatment of the Indonesian childhood ALL protocol. Patients were randomized to the ciprofloxacin arm (n = 58) and to the placebo arm (n = 52). Oral ciprofloxacin monotherapy or oral placebo was administered twice a day. All events during induction were recorded: toxic death, abandonment, resistant disease, and complete remission rate. Of 110 patients enrolled in this study, 79 (71.8%) achieved CR. In comparison to the placebo arm, the ciprofloxacin arm had lower nadir of absolute neutrophil count during induction with median of 62 (range: 5-884) versus 270 (range: 14-25,480) × 10(9) cells/L (P < 0.01), greater risks for experiencing fever (50.0% versus 32.7%, P = 0.07), clinical sepsis (50.0% versus 38.5%, P = 0.22), and death (18.9% versus 5.8%, P = 0.05). In our setting, a reduced intensity protocol in a low-income situation, the data warn against using ciprofloxacin prophylaxis during induction treatment. A lower nadir of neutrophil count and higher mortality were found in the ciprofloxacin group.
  Hematology Research and Reviews 01/2013; 4:1-9.
• Article: Barriers and perceived limitations to early treatment of hemophilia.

  Kapil Saxena
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  ABSTRACT: Early treatment of bleeds in hemophilia patients, both with and without inhibitors, has been shown to be of immense benefit in the overall clinical outcome. Despite the advantages of treating the bleeding episodes early, significant barriers and limitations remain. The aim of this review is to highlight the various barriers and perceived limitations to early therapy of bleeding episodes, especially in patients who have developed inhibitors to factor VIII. The peer-reviewed literature was searched for articles on hemophilia patients, with and without inhibitors, and early treatment, to identify the barriers to early treatment and potential impact on patient outcomes. The most important barrier is the educational barrier, which involves lack of awareness among patients regarding the signs of a bleed, as well as importance of early therapy. It is also common for parents or caregivers of school-age children to exhibit inconvenience and scheduling barriers. Distance to the treatment center can also play a role here. Some patients experience financial barriers related to cost of clotting factor products, insurance coverage, or insurance caps and out-of-pocket costs. Rarely, there can also be problems related to venous access or home infusion. Lastly, multiple psychosocial barriers can prevent adherence to treatment regimens. Identification and addressing these individual barriers will result in improved compliance rates, prevent joint damage, be more cost-effective, and lead to better overall health of these patients.
  Hematology Research and Reviews 01/2013; 4:49-56.
• Article: Severe hypernatremia and hyperchloremia in an elderly patient with IgG-kappa-type multiple myeloma.

  Shinsaku Imashuku, Naoko Kudo, Kagekatsu Kubo
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  ABSTRACT: A 77-year-old male was admitted to hospital after suffering a pelvic bone fracture in a road traffic accident and was incidentally found to have IgG-kappa-type multiple myeloma with hypercalcemia. The patient was also noted to be hypokalemic and had low HCO3 (-), with possible damage to the distal tubules in the kidneys. When the treatment was begun with bortezomib/dexamethasone/elcatonin and sodium bicarbonate (NaHCO3) in normal saline (equivalent to a daily sodium dose of 200 millimoles per liter [mmol/L]), the patient was in a state of poor oral fluid intake. The patient developed hypernatremia and hyperchloremia, with a peak serum sodium and chloride levels of 183 mmol/L and 153 mmol/L, respectively, at the sixth day after the start of treatment. Following the switch of the intravenous infusions from normal saline to soldem 1 and soldem 3 solutions, these high-electrolyte levels gradually returned to normal over the next 7 days. Although the patient showed disturbed consciousness (Japan Coma Scale = JCS-I-3) during the period of electrolyte abnormality, he eventually fully recovered without sequelae. In this patient, we successfully managed the severe hypernatremia/hyperchloremia, caused by the combined effects of intravenous saline burden in a state of poor oral fluid intake, during the treatment for IgG-kappa type multiple myeloma.
  Hematology Research and Reviews 01/2013; 4:43-47.
• Article: Treatments for chronic myeloid leukemia: a qualitative systematic review.

  Roxanne Ferdinand, Stephen A Mitchell, Sarah Batson, Indra Tumur
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  ABSTRACT: Chronic myeloid leukemia (CML) is a myeloproliferative disorder of blood stem cells. The tyrosine kinase inhibitor (TKI) imatinib was the first targeted therapy licensed for patients with chronic-phase CML, and its introduction was associated with substantial improvements in response and survival compared with previous therapies. Clinical trial data are now available for the second-generation TKIs (nilotinib, dasatinib, and bosutinib) in the first-, second-, and third-line settings. A qualitative systematic review was conducted to qualitatively compare the clinical effectiveness, safety, and effect on quality of life of TKIs for the management of chronic-, accelerated-, or blast-phase CML patients. Included studies were identified through a search of electronic databases in September 2011, relevant conference proceedings and the grey literature. In the first-line setting, the long-term efficacy (up to 8 years) of imatinib has been confirmed in a single randomized controlled trial (International Randomized Study of Interferon [IRIS]). All second-generation TKIs reported lower rates of transformation, and comparable or superior complete cytogenetic response (CCyR), major molecular response (MMR), and complete molecular response rates compared with imatinib by 2-year follow-up. Each of the second-generation TKIs was associated with a distinct adverse-event profile. Bosutinib was the only second-generation TKI to report quality-of-life data (no significant difference compared with imatinib treatment). Data in the second- and third-line setting confirmed the efficacy of the second-generation TKIs in either imatinib-resistant or -intolerant patients, as measured by CCyR and MMR rates. Data from first-line randomized controlled trials reporting up to 2-year follow-up indicate superior response rates of the second-generation TKIs compared with imatinib. Current evidence from single-arm studies in the second-line setting confirm that nilotinib, dasatinib, and bosutinib are valuable treatment options for the significant subgroup of patients who are intolerant or resistant to imatinib treatment.
  Hematology Research and Reviews 01/2012; 3:51-76.
• Article: Letter to the editor.

  Vito D'Alessandro, Mario Greco
  Hematology Research and Reviews 01/2012; 3:43-4.
• Source

  Available from: PubMed Central

  Article: Preventing stroke in atrial fibrillation patients - clinical utility of oral anticoagulants.

  Manish B Jhawar, Greg Flaker
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  ABSTRACT: Atrial fibrillation is the most common of the cardiac arrhythmias and is associated with high risk of stroke and systemic thromboembolism. Prevention of these complications is therefore a major component of clinical management in patients with this rhythm disorder. The choice of antithrombotic therapy in any given patient depends on his or her risk profile and needs to be carefully balanced against the risk of bleeding. In this review we discuss the pathophysiology of thrombogenesis in atrial fibrillation, risk factors for systemic thromboembolism in atrial fibrillation, patient risk stratification modules both for systemic thromboembolism and the risk of bleeding, current antithrombotic therapy strategies, clinicoepidemiological evidence that led to their evolvement, the challenges that plague them, recent developments in the field and how they could possibly affect our future clinical decision making.
  Hematology Research and Reviews 01/2012; 3:1-13.
• Article: Spontaneous resolution of macrocytic anemia: old disease revisited.

  Shinsaku Imashuku, Naoko Kudo, Shigehiro Kaneda
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  ABSTRACT: We report here on a 60-year-old male with alcohol-related macrocytic anemia. He was hospitalized on three occasions with hemoglobin < 9.0 g/dL and mean corpuscular volume > 130 fL. Careful history taking and observation of his blood status led us to make a correct diagnosis. At the time of each of his admissions, only with bed rest and abstinence from alcohol did our patient dramatically show spontaneous recovery of anemia in association with a rapid decline of serum γ-glutamyl transpeptidase values. It is well recognized that marrow abnormalities in alcoholic patients are reversible. Physicians should be aware that there is a subset of patients with macrocytic anemia that could be improved without medication.
  Hematology Research and Reviews 01/2012; 3:45-7.
• Article: Optimal management of pernicious anemia.

  Emmanuel Andres, Khalid Serraj
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  ABSTRACT: Pernicious anemia (also known as Biermer's disease) is an autoimmune atrophic gastritis, predominantly of the fundus, and is responsible for a deficiency in vitamin B12 (cobalamin) due to its malabsorption. Its prevalence is 0.1% in the general population and 1.9% in subjects over the age of 60 years. Pernicious anemia represents 20%-50% of the causes of vitamin B12 deficiency in adults. Given its polymorphism and broad spectrum of clinical manifestations, pernicious anemia is a great pretender. Its diagnosis must therefore be evoked and considered in the presence of neurological and hematological manifestations of undetermined origin. Biologically, it is characterized by the presence of anti-intrinsic factor antibodies. Treatment is based on the administration of parenteral vitamin B12, although other routes of administration (eg, oral) are currently under study. In the present update, these various aspects are discussed with special emphasis on data of interest to the clinician.
  Hematology Research and Reviews 01/2012; 3:97-103.
• Source

  Available from: PubMed Central

  Article: 7-Nitroindazole and its rapidly emerging role in opioid pain management and withdrawal.

  Shailendra Kapoor
  Hematology Research and Reviews 01/2012; 3:15-6.
• Article: First-line treatment of chronic myeloid leukemia with nilotinib: critical evaluation.

  Pier Paolo Piccaluga, Stefania Paolini, Clara Bertuzzi, Antonio De Leo, Gianantonio Rosti
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  ABSTRACT: The therapeutic landscape of chronic myeloid leukemia (CML) has changed dramatically in the last decade. In particular, the availability of imatinib mesylate, a tyrosine kinase inhibitor targeting BCR-ABL, has led to profound and durable remissions in the majority of patients. However, a couple of issues have emerged and partially obscured this scenario. First, it has become clear that a significant proportion of patients either present with primary resistance to imatinib or develop secondary resistance sooner or later during treatment. Second, although the drug is generally well tolerated, a percentage of patients eventually cease treatment because of toxicity. Bearing this in mind, second-generation tyrosine kinase inhibitors have been introduced, including nilotinib. Phase I and II studies indicate remarkable activity for this compound in CML cases resistant to imatinib, including some of those carrying BCR-ABL1 mutants. More recently, two Phase II studies and a III randomized Phase clinical trial demonstrated the superiority of nilotinib compared with imatinib in terms of complete cytogenetic and major molecular responses, which are two relevant surrogate measures of long-term survival in CML. In this paper, we review the most relevant data on nilotinib as first-line treatment for CML, and discuss the rationale for its routine use, as well as some possible future perspectives for CML patients.
  Hematology Research and Reviews 01/2012; 3:151-6.
• Article: Rituximab for managing acquired hemophilia A in a case of chronic neutrophilic leukemia with the JAK2 kinase V617F mutation.

  Shinsaku Imashuku, Naoko Kudo, Kagekatsu Kubo, Katsuyasu Saigo, Nanako Okuno, Kaoru Tohyama
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  ABSTRACT: Acquired hemophilia A is rarely found in association with myeloproliferative neoplasms, such as the JAK2 kinase V617F mutation-positive chronic neutrophilic leukemia (CNL). An 80-year-old Japanese male was diagnosed with acquired hemophilia A. He had compartment-like symptoms due to soft tissue hemorrhage in his left forearm and right lower extremity. A blood examination showed neutrophilia with a white blood cell count of 31,900/μL (91.9% neutrophils), an activated partial thromboplastin time of 69.0 seconds, coagulation factor VIII (FVIII) < 1.0%, and anti-FVIII inhibitor, 190 BU/mL. The bleeding episodes were controlled with intravenous activated prothrombin complex concentrate (FEIBA(®)) followed by recombinant factor VIIa (NovoSeven(®)). In addition, oral prednisolone (maximum dose, 30 mg/day) plus four doses of rituximab effectively suppressed anti-FVIII inhibitor levels while simultaneously reducing the neutrophil count. CNL with the JAK2 kinase V617F mutation was identified as the underlying disease. This report describes the effectiveness of a combination of prednisolone and rituximab in managing acquired hemophilia A in an elderly man with a rare case of JAK2 kinase V617F mutation-positive CNL.
  Hematology Research and Reviews 01/2012; 3:157-61.