Transfusion in the Patient With Sickle Cell Disease: A Critical Review of the Literature and Transfusion Guidelines

Transfusion Medicine Program, Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, GA, USA.
Transfusion Medicine Reviews (Impact Factor: 2.92). 04/2007; 21(2):118-33. DOI: 10.1016/j.tmrv.2006.11.003
Source: PubMed


The clinical outcomes of sickle cell disease (SCD) have vastly improved over the years in great part as a result of advanced medical technologies, improved patient education, and multidisciplinary care. A key component in the successful management of patients with SCD is red blood cell transfusion therapy used in the treatment and prevention of sickle cell complications. However, although the successful application of transfusion therapy has significantly improved the morbidity and mortality of patients with SCD, the literature that addresses the appropriate selection and use of blood products continues to evolve with no clear universal standard of care. Our objectives were to provide an in-depth review of the current literature on transfusion therapy in SCD and to provide a set of guidelines for the transfusion management of patients with SCD.

Download full-text


Available from: Cassandra D Josephson, Feb 10, 2015
  • Source
    • "[5] However, because all of these therapies have significant morbidity, caution is appropriately applied with any such treatment decisions. [6]–[8] Ongoing efforts to identify infants and children at high risk for severe disease are especially relevant for choosing treatment options. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Among older children with sickle cell anemia, leukocyte counts, hemoglobin, and reticulocytosis have previously been suggested as disease severity markers. Here we explored whether these blood parameters may be useful to predict early childhood disease severity when tested in early infancy, defined as postnatal ages 60-180 days. Data from fifty-nine subjects who were followed at Children's National Medical Center's Sickle Cell Program for at least three years was retrospectively analyzed. Comparisons were made between white blood cell counts, hemoglobin and reticulocyte levels measured at ages 60-180 days and the clinical course of sickle cell anemia during infancy and childhood. A majority of subjects had demonstrable anemia with increased reticulocytosis. Only increased absolute reticulocyte levels during early infancy were associated with a significant increase in hospitalization during the first three years of life. Higher absolute reticulocyte counts were also associated with a markedly shorter time to first hospitalizations and a four-fold higher cumulative frequency of clinical manifestations over the first three years of life. No significant increase in white blood cell counts was identified among the infant subjects. These data suggest that during early infancy, increased reticulocytosis among asymptomatic SCA subjects is associated with increased severity of disease in childhood.
    Full-text · Article · Aug 2013 · PLoS ONE
  • Source
    • "In concordance with these findings, the majority of survey respondents did not routinely transfuse for uncomplicated pregnancy. Published recommendations do not identify acute painful episodes without complications as an indication for transfusion [10, 12, 14]. In our survey, 9% of the sample indicated this was “always” an indication, whereas the combination of rarely and never constituted 50% of the responses. "
    [Show abstract] [Hide abstract]
    ABSTRACT: The purpose of this study was to characterize transfusion practices in the management of sickle cell disease and to identify factors attributing to differences in prescribing practices among Florida hematologists/oncologists. A cross-sectional study was performed in 2005-2006 utilizing a mail survey. The survey instrument addressed practice characteristics, sickle cell patient populations, transfusion settings, indications and techniques, red blood cell phenotype specifications/modifications, use of practice guidelines, and educational resource utilization. One hundred fifty two physicians (75% adult-oriented, 25% pediatric) completed the survey. Non-academic practice settings (78 %) were the primary location. Pediatric practices had a larger percentage of patients with overt strokes, and receiving hydroxyurea therapy than adult-oriented practices. The majority of survey respondents did not request limited phenotypically matched red blood cells on a routine basis. The majority of pediatric practices (60%) had individually defined transfusion practice guidelines in contrast to 8% of adult-oriented practices. There were statistically significant differences for pediatric and adult-oriented practices in managing certain acute and chronic transfusion indications. Analysis of clinical vignette data revealed variation among hematologists/oncologists in the transfusion management of common clinical scenarios. The study underscores the need for the development and dissemination of comprehensive sickle cell transfusion guidelines and protocols.
    Full-text · Article · Dec 2012
  • Source
    • "Blood transfusions are currently the most studied and accepted therapy for those patients. Chronic red blood cell (RBC) exchanges are effective in preventing the recurrence of strokes or VOC (Wayne et al, 1993; Pegelow et al, 1995; Adams et al, 1998; Miller et al, 2001; Josephson et al, 2007) while simple blood transfusions are usually used in case of severe anaemia or ACS (Hirani et al, 2011). For chronic and acute transfusions, the goal is to increase the oxygen carrying capacity and to dilute abnormal SS-RBCs in whole blood. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Patients with sickle cell disease (SCD) can present several severe symptoms during their lifetime, including painful events due to vascular occlusion (VOC). Even though multiple factors are involved in VOC, hypoxia is the most important triggering factor. Inositol hexaphosphate (IHP) reduces the oxygen-haemoglobin affinity thus improving the oxygen release in the blood stream and in the tissues. Thus, IHP-loaded homologous red blood cells (IHP-RBCs) could be able to reduce disorders in SCD. The effectiveness of treatment was assessed in two types of SCD transgenic mice (BERK and SAD). The administration of four repeated injections of IHP-RBCs in BERK mice resulted in an improved survival rate and brain development, prevention of severe anaemia and a greatly lowered risk of VOC. After one injection of IHP-RBCs, SAD mice were subjected to acute hypoxic stress. Analysis of the lungs revealed significantly decreased mRNA levels of molecules involved in intravascular disorders. Our results showed that transfusion of homologous IHP-RBCs, by increasing the oxygen delivery, reduces SCD disorders in sickle transgenic mice.
    Full-text · Article · Mar 2012 · British Journal of Haematology
Show more