Singer S T, Wu V, Mignacca R, Kuypers F A, Morel P, Vichinsky E P
Department of Hematology/Oncology at the Children's Hospital Oakland, California, USA.
Blood. 2000 Nov 15;96(10):3369-73.
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.
溶血性同种抗体和红细胞自身抗体的产生使地中海贫血患者的输血治疗变得复杂。我们评估了64例接受输血的地中海贫血患者（75%为亚洲人）中这种现象的发生率、原因及预防措施。确定了供者（大多为白人）与亚洲受者之间红细胞（RBC）表型差异对同种免疫发生率的影响。还研究了额外的输血因素和患者免疫因素。64例患者（75%为亚洲人）中有14例（22%）发生了同种免疫。在构成主要供者库的白种人与亚洲受者之间，发现K、c、S和Fyb抗原的RBC表型不匹配，这占亚洲患者同种抗体的38%。行脾切除术的患者同种免疫发生率高于未行脾切除术的患者（36%对12.8%；P = 0.06）。通过抗人球蛋白试验阳性确定，64例患者中有25%即16例产生了红细胞自身抗体，其中16例中有3例因此发生了严重溶血性贫血。在这16例中，11种抗体被鉴定为免疫球蛋白G [IgG]型，5种为IgM型。自身免疫与同种免疫以及无脾有关（分别为44%和56%）。输注的RBC变形能力异常，在无脾患者中更明显，这可能刺激了抗体产生。与标准ABO - D系统表型匹配的血液（白细胞去除率为60%）相比，输注Rh和Kell系统表型匹配的血液（白细胞去除率为92%）被证明可有效预防同种免疫（2.8%对33%；P = 0.0005）。同种免疫和自身免疫是亚洲地中海贫血患者常见的严重并发症，这些患者受到供者 - 受者RBC抗原不匹配和免疫因素的影响。
