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Blood, 15 November 2000, Vol. 96, No. 10, pp. 3369-3373
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Alloimmunization and erythrocyte autoimmunization in
transfusion-dependent thalassemia patients of predominantly
Asian descent
Sylvia T. Singer,
Vivian Wu,
Robert Mignacca,
Frans A. Kuypers,
Phyllis Morel, and
Elliott P. Vichinsky
From the Department of Hematology/Oncology at the
Children's Hospital Oakland, the Children's Hospital Oakland Research
Institute, and the American Red Cross Blood Services, Northern
California Region, Oakland, CA, and the Department of
Hematology/Oncology, Valley Children's Hospital, Madera, CA.
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.
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Related Letter in Blood Online:
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Alloimmunization in Hong Kong southern Chinese transfusion-dependent thalassemia patients
- Hok-Kung Ho, Shau-Yin Ha, Chun-Kit Lam, Godfrey C. F. Chan, Tsz-Leung Lee, Alan K. S. Chiang, Yu-Lung Lau, and Sylvia T. Singer
Blood 2001 97: 3999-4000.
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