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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Red blood cells (RBC) transfusion is critical in managing acute and chronic complications in sickle cell disease (SCD); however, it is complicated by RBC alloimmunization, iron overload, transfusion reactions and infection. Several reports documented an increased incidence of alloantibodies in transfused individuals with SCD, especially for Rh and Kell antigens. As a result, the National Institutes of Health Expert Panel and British Society for Haematology guidelines recommend primary matching for C/c, E/e and K antigens in addition to ABO/RhD for RBC transfusions. However, the evidence supporting these recommendations was cited as limited and understanding of alloimmunization in SCD is evolving. To examine the limitations of the evidence, we undertook a systematic review of evidence behind recommendations for limited and extended serologic and genotypic RBC antigen matching to reduce alloimmunization, autoimmunization and transfusion reactions. Searches of PubMed, Embase, Cochrane, and Web of Science databases using MeSH index and free text terms between 1976 through October 2015 and papers and captured through July 2016 through review references in papers, word of mouth, and ongoing Google Scholar and Medline Alerts identified 303 unique articles. Nineteen articles met inclusion criteria and were classified by the Oxford Centre Evidence Based levels of evidence. Strengthening the Reporting of Observational Studies in Epidemiology checklists were completed for 18 of the 19 studies. There were no prospective randomized controlled trials. Sixteen of the articles were cohort studies, two were cross-sectional studies, and one decision tree model examining costs. Low-quality evidence from observational cohort studies supports that alloimmunization prevalence can be decreased by extending serological RBC antigen matching. Transfusion reactions are generally poorly and inconsistently reported. There was no evidence reporting the effect prophylactic genotypic matching has on alloimmunization, autoimmunization or transfusion reactions. There were no studies comparing prophylactic genotypic matching to serologic matching. High-quality evidence was lacking to support clinical decision making regarding best transfusion practices. Multicenter prospective randomized clinical trials are needed to determine best strategies for reducing the rate of alloimmunization using serologic and genotypic matching.
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PMID:Impact of Red Blood Cell Antigen Matching on Alloimmunization and Transfusion Complications in Patients with Sickle Cell Disease: A Systematic Review. 3012 66

Sickle cell disease (SCD) is the most common monogenic disorder in sub-Saharan Africa (SSA). Blood transfusion to increase the oxygen carrying capacity of blood is vital in the management of many patients with SCD. However, red blood cell (RBC) alloimmunization is a major challenge to transfusions in these patients. Commonly in SSA, pretransfusion tests only involve ABO D grouping and compatibility without RBC antibody testing. Data on the frequency of RBC alloimmunization in patients with SCD in SSA are limited. We performed a systematic review and meta-analysis on available data on alloimmunization in transfused patients with SCD to determine the published prevalence of RBC alloimmunization in SCD patients in SSA. Six databases were systematically searched to identify relevant studies, without year or language restrictions. In all, 249 articles were identified and 15 met our selection criteria. The overall proportion of alloimmunization was 7.4 (95% confidence interval: 5.1-10.0) per 100 transfused patients. Antibodies against E, D, C, and K antigens accounted for almost half of antibody specificities, and antibodies to low- and high-frequency antigens were also common and represented almost 30% (20% to low-frequency antigens and 9% to high-frequency antigens) of specificities. Heterogeneity between studies was moderate, and meta-analysis found region of Africa as the major contributor to the heterogeneity. We also observed inconsistencies across studies in reporting of factors that may influence alloimmunization. This review provides an overview of the extent of the alloimmunization problem in SSA and provides a baseline against which to compare the effect of any interventions to reduce the alloimmunization risk.
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PMID:Red Blood Cell Alloimmunization in Transfused Patients With Sickle Cell Disease in Sub-Saharan Africa; a Systematic Review and Meta-Analysis. 3134 90

Erythrocytapheresis, red blood cell (RBC) depletion, and RBC exchange transfusions are apheresis techniques used to rapidly lower the circulating RBC mass or to exchange the patient erythrocyte mass with donor RBC. Automated RBC exchange is performed using an apheresis device, while manual RBC exchange is based on sequential phlebotomies and isovolemic replacement. Compared to simple RBC transfusions, RBC exchange offers several advantages, e.g., a lower risk for iron accumulation and efficient control of pathological erythrocyte populations. Disadvantages are the higher costs of the procedure, the increased use of donor RBC, and the requirement of apheresis devices and trained hospital staff. The most frequent indication for RBC exchange is sickle cell disease (SCD). RBC exchange transfusions are standard treatment in SCD patients with a history of or a risk for acute stroke and are clinical options for other acute complications of SCD. The most common indication for RBC depletion is the removal of donor RBC from the bone marrow grafts in major ABO-incompatible allogeneic hematopoietic stem cell transplantation to avoid immediate hemolysis. Rare indications for RBC exchange are severe infections with intraerythrocytic pathogens such as malaria or babesiosis and severe erythrocytosis or hereditary hemochromatosis where the aim is to rapidly decrease RBC populations or the iron content. However, only few high-quality studies are available looking at the efficacy of RBC exchange in the different disease entities, and treatment is often based on low levels of evidence and should therefore be decided in close collaboration with a transfusion medicine specialist.
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PMID:Red Blood Cells: Exchange, Transfuse, or Deplete. 3193 70

Laboratory and epidemiologic studies have clarified how persons born with malaria-resistant red blood cells (RBCs)-like group-O, sickle-trait, and C-trait RBCs-are protected against death or severe disease due to Plasmodiumfalciparum (Pf) infection. Compared to malaria-promoting RBCs-like non-O or hemoglobin-AA RBCs-inborn RBC protection against severe Pf malaria can be profound: up to 10-fold greater. Given that "the Berlin patient" success showed patients do not have to be born with disease-resistant cells to benefit from them, why have the biologically plausible benefits of exchange transfusion (ET) of malaria-resistant RBCs not yet been evaluated? Unfortunately, a 2013 ET-for-malaria meta-analysis could not quantify the impact on mortality of ET of malaria-resistant RBCs because RBC malaria resistance variables (ABO group, hemoglobin type, enzyme levels, etc.) had not been reported in any of the ET studies used in that meta-analysis. To promote evaluation of the therapeutic impact of specific malaria-resistant RBCs, we urge clinicians to always report ABO blood group (and all other RBC malaria-resistance variables they are aware of) when they use ET to rescue Pf-infected patients. Prudent selection of donor RBCs has successfully optimized ET for sickle cell disease patients, and this precedent suggests selection of special malaria-resistant donor RBCs may optimize ET for Pf-malaria patients. Given that ET is used worldwide as a rescue adjunct, we feel it is most prudent to now assume-until proven otherwise-that considering and reporting the Pf-malaria-resistance variables of the RBCs to be transfused-at least ABO status-will help optimize ET-for-malaria.
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PMID:ABO blood group should be considered and reported when red blood cell exchange transfusion is used to treat Plasmodiumfalciparum Malaria patients. 3236 95

Red blood cell alloimmunisation after transfusion of red blood cell concentrates carries a risk for every recipient. This risk is particularly high for patients with conditions such as sickle cell disease. However, red blood cell alloimmunisation can also occur after platelet concentrate transfusion. All blood group systems other than ABO are affected, and there are several mechanisms responsible for this alloimmunisation. The practical implications of this are a need to match red blood cell concentrates in all alloimmunised patients and, in pregnant women, recongnition of the risk of developing haemolytic disease of the foetus and newborn. Several measures can be taken to prevent alloimmunisation: in the case of the D antigen, for example, anti-RhD immunoglobulins can be infused before transfusing platelet concentrates from an RhD-positive donor in a RhD-negative recipient.
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PMID:Red blood cell alloimmunisation after platelet transfusion (excluding ABO blood group system). 3254 26


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