Experience with donors matched for minor blood group antigens in patients with sickle cell anemia who are receiving chronic transfusion therapy

Ambruso, Daniel R.; Githens, John H.; Alcorn, Roosevelt; Dixon, Danielle; Brown, Lorraine; Vaughn, W M; Hays, Taru

doi:10.1046/j.1537-2995.1987.27187121485.x

Cited by 136 publications

(118 citation statements)

References 9 publications

(1 reference statement)

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“…Although randomized controlled trials have not been performed, extended red cell antigen-matching has been shown in numerous single institutional and prospective multicenter experiences to significantly reduce the incidence of alloantibody production in SCD. [28][29][30] Data suggest a 40% to 90% reduction, depending on the extent of antigen matching (4 to 15 antigens), accompanied by decreased DHTRs compared with historical rates in patients with SCD. Despite this success, some institutions perform phenotype matching only after the patient develops the first alloantibody.…”

Section: Clinical Considerations For Patients With Scdmentioning

confidence: 99%

Molecular biology of the Rh system: clinical considerations for transfusion in sickle cell disease

Chou

Westhoff²

2009

Hematology

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The last decade has witnessed an abundance of information detailing the genetic diversity of the RH locus which has exceeded all estimates predicted by serology. Well over 120 RHD and over 60 different RHCE alleles have been documented, and new alleles are still being discovered. For clinical transfusion medicine, RH genetic testing can now be used to determine RHD zygosity, resolve D antigen status, and detect altered RHD and RHCE genes in individuals at risk for producing antibodies to high-incidence Rh antigens, particularly patients with sickle cell disease (SCD).T ransfusion of patients with sickle cell disease (SCD) represents a significant challenge in clinical transfusion medicine with red blood cell (RBC) alloimmunization a primary and serious complication of transfusions. A major cause of alloimmunization in patients with SCD is the disparate distribution of red cell antigens between donors, who are primarily of European ancestry, and patients with SCD, who are primarily of African ancestry. Management of alloimmunization in SCD has been the subject of much debate, 1,2 and currently there is no standard approach. Over two thirds of alloantibodies formed by patients with SCD have Rh blood group specificities. Many programs transfuse patients with SCD with RBCs that are phenotype-matched for D, C/c, E/e, and K, and some also supply RBCs from African-American donors when possible. Although these approaches reduce the incidence of alloantibody production, patients still become alloimmunized. Genetic analysis of patients who develop antibodies in the face of conventional antigen matching has revealed that these patients carry altered RH alleles. RH Genes and Rh ProteinsTwo genes, RHD and RHCE, lie in close proximity on chromosome 1 and encode 416-amino acid Rh proteins: one encodes the D antigen, and the other encodes CE antigens in various combinations (ce, cE, Ce, or CE) (Figure 1). Each gene has ten exons; they are 97% identical and encode proteins that differ by 32 to 35 amino acids (Figure 1). This contrasts with most blood group antigens, which are encoded by single genes with alleles that differ TRANSFUSION MEDICINE ___________________________________________________________________________________ by only one or a few amino acids. For comprehensive reviews, see Westhoff 3 and Avent. The conventional RH genes shown in Figure 1 are found in all population groups, although with different frequencies. Commercial antibody reagents detect expression of the five principal Rh antigens: D, C, c, E, and e. Variant RHD and RHCE alleles encode Rh proteins with amino acid changes that cannot be distinguished serologically, but can be recognized by the immune system as foreign. Of relevance for transfusion in patients with SCD, RH alleles encoding altered C and e antigens are frequent in African ethnic groups. 5,6 Some patients with SCD are at risk for production of antibodies to both the RhCE and RhD proteins, a serious and potentially life-threatening complication. Variations of RhD

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Section: Clinical Considerations For Patients With Scdmentioning

confidence: 99%

Molecular biology of the Rh system: clinical considerations for transfusion in sickle cell disease

Chou

Westhoff²

2009

Hematology

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“…The RH haplotype Dce (R o ) is most prevalent in Africans (44 %). Ambruso et al have demonstrated that prevention of alloimmunization by supplying of phenotype-specific red cell is an approach, which is cost effective and leads to a tenfold decrease in alloantibody formation [9,10].…”

Section: Discussionmentioning

confidence: 99%

Fatal Delayed Haemolytic Transfusion Reaction and Hyperhaemolysis Syndrome in a Pregnant Woman with Sickle Cell Anaemia

Asnawi

Sathar

Mohamed

et al. 2015

Indian J Hematol Blood Transfus

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Clinical manifestations of sickle cell disease (SCD) arise from the tendency of the sickle haemoglobin to polymerize and deform red blood cells into the characteristic sickle shape. Sickle cell crisis is a devastating complication that may occur in patients with SCD. If not managed properly permanent organ damage and even death may be the final outcome. A case of a 32-year-old Nigerian lady, Gravida 1 Para 0 in her first trimester, with SCD who developed signs and symptoms of delayed haemolytic transfusion reaction after receiving packed red cell transfusion is demonstrated. Multiple red cell alloantibodies were detected in the patient's plasma; anti-Fy a, anti-Jk b and anti-E. The patient miscarriaged and succumbed to complications of hyperhaemolysis with delayed haemolytic transfusion reaction, acute chest syndrome and renal failure. There is an urgent need for mandatory red cell antibody screen and identification especially in high-risk cases. Prevention of alloimmunization by supplying phenotype-specific red cells is also required.

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“…In patients with SCD receiving only ABO-and D-matched RBCs, the rate of alloimmunisation ranges from 18% to as high as 76%. 47,48,52,[55][56][57][58][59] The exact rate is dependent on many factors, including the extent of RBC antigen matching, the degree of homogeneity between the recipients' and donors' ethnic backgrounds and the frequency of transfusion. The Rh and anti-K antibodies comprise over two-thirds of the RBC antibodies encountered.…”

Section: 43mentioning

confidence: 99%

Transfusion in Haemoglobinopathies: Review and recommendations for local blood banks and transfusion services in Oman

Al-Riyami

Daar

2018

Sultan Qaboos Univ Med J

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Sickle cell disease and homozygous β-thalassaemia are common haemoglobinopathies in Oman, with many implications for local healthcare services. The transfusions of such patients take place in many hospitals throughout the country. Indications for blood transfusions require local recommendations and guidelines to ensure standardised levels of care. This article summarises existing transfusion guidelines for this group of patients and provides recommendations for blood banks and transfusion services in Oman. This information is especially pertinent to medical professionals and policy-makers developing required services for the standardised transfusion support of these patients.

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Experience with donors matched for minor blood group antigens in patients with sickle cell anemia who are receiving chronic transfusion therapy

Cited by 136 publications

References 9 publications

Molecular biology of the Rh system: clinical considerations for transfusion in sickle cell disease

Molecular biology of the Rh system: clinical considerations for transfusion in sickle cell disease

Fatal Delayed Haemolytic Transfusion Reaction and Hyperhaemolysis Syndrome in a Pregnant Woman with Sickle Cell Anaemia

Transfusion in Haemoglobinopathies: Review and recommendations for local blood banks and transfusion services in Oman

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