title: Alloimmunization and erythrocyte autoimmunization in transfusion-dependent thalassemia patients of predominantly asian descent.
abstract: The development of hemolytic alloantibodies and erythrocyte autoantibodies complicates transfusion therapy in thalassemia patients. The frequency, causes, and prevention of this phenomena among 64 transfused thalassemia patients (75% Asian) were evaluated. The effect of red blood cell (RBC) phenotypic differences between donors (mostly white) and Asian recipients on the frequency of alloimmunization was determined. Additional transfusion and patient immune factors were examined. 14 (22%) of 64 patients (75% Asian) became alloimmunized. A mismatched RBC phenotype between the white population, comprising the majority of the donor pool, and that of the Asian recipients, was found for K, c, S, and Fyb antigens, which accounts for 38% of the alloantibodies among Asian patients. Patients who had a splenectomy had a higher rate of alloimmunization than patients who did not have a splenectomy (36% vs 12.8%; P =.06). Erythrocyte autoantibodies, as determined by a positive Coombs test, developed in 25% or 16 of the 64 patients, thereby causing severe hemolytic anemia in 3 of 16 patients. Of these 16, 11 antibodies were typed immunoglobulin G [IgG], and 5 were typed IgM. Autoimmunization was associated with alloimmunization and with the absence of spleen (44% and 56%, respectively). Transfused RBCs had abnormal deformability profiles, more prominent in the patients without a spleen, which possibly stimulated antibody production. Transfusion of phenotypically matched blood for the Rh and Kell (leukodepleted in 92%) systems compared to blood phenotypically matched for the standard ABO-D system (leukodepleted in 60%) proved to be effective in preventing alloimmunization (2.8% vs 33%; P =.0005). Alloimmunization and autoimmunization are common, serious complications in Asian thalassemia patients, who are affected by donor-recipient RBC antigen mismatch and immunological factors.
authors.affiliation: Department of Hematology/Oncology at the Children's Hospital Oakland, California, USA.
authors.forename: S T
authors.initials: ST
authors.lastname: Singer
authors.forename: V
authors.initials: V
authors.lastname: Wu
authors.forename: R
authors.initials: R
authors.lastname: Mignacca
authors.forename: F A
authors.initials: FA
authors.lastname: Kuypers
authors.forename: P
authors.initials: P
authors.lastname: Morel
authors.forename: E P
authors.initials: EP
authors.lastname: Vichinsky
mesh_terms: D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000209:etiology / Q000276:immunology; D044466:Asian People; D001323:Autoantibodies / Q000032:analysis / Q000097:blood*; D001782:Blood Donors; D001803:Blood Transfusion / Q000379:methods; D002648:Child; D002675:Child, Preschool; D004907:Erythrocyte Deformability / Q000276:immunology; D004912:Erythrocytes / Q000276:immunology* / Q000473:pathology; D005260:Female; D006446:Hemoglobin E / Q000276:immunology; D006455:Hemoglobins, Abnormal / Q000276:immunology; D006801:Humans; D015994:Incidence; D007518:Isoantibodies / Q000032:analysis / Q000097:blood*; D008297:Male; D010641:Phenotype; D011247:Pregnancy; D013789:Thalassemia / Q000150:complications / Q000276:immunology* / Q000628:therapy; D065227:Transfusion Reaction; D044465:White People; D017085:alpha-Thalassemia / Q000276:immunology / Q000473:pathology
chemical_list: D001323:Autoantibodies; D006455:Hemoglobins, Abnormal; D007518:Isoantibodies; D006446:Hemoglobin E; C035541:Hemoglobin Constant Spring