Seferi I, Xhetani M, Face M, Burazeri G, Nastas E, Vyshka G
National Blood Transfusion Centre, Tirana, Albania.
Faculty of Natural Sciences, University of Tirana, Tirana, Albania.
Int J Lab Hematol. 2015 Aug;37(4):569-74. doi: 10.1111/ijlh.12362. Epub 2015 Apr 11.
Thalassemia major is a common hemoglobinopathy in Albania. However, there are no data available on the frequency of RBC alloimmunization and autoimmunization in transfusion-dependent Albanian patients with thalassemia.
A total of 118 patients with thalassemia receiving regular transfusions were studied during 5 years with antibody screening. During this period, they were exclusively transfused with blood matched for ABO, Rhesus and Kell system. These patients were previously exposed to unmatched blood because of blood shortages.
Fourteen of 118 (11, 8%) patients developed alloantibodies. Twelve (10, 1%) were already present at the start of the study. Only 2 (1, 7%) were formed after the application of a strict Rh and Kell matching policy. The most common antibody was anti-K, followed by anti-D, anti-C, anti-E, anti-c, anti-e, anti-Jk(b) , and anti-C(w) . Three patients developed anti-D plus anti-C. Anti-K was combined with Rh antibodies in two of five cases. Anti-c was combined with anti-E in two of three cases. The majority of antibodies (10/14) belonged to the Rh blood group system. With the exception of the anti-Jk(b) and the anti-C(w) , all antibodies were already present at the beginning of the follow-up period. During our follow-up, 27 patients (22.8%) developed autoantibodies. A strong coincidence was found between the presence of alloantibodies and autoantibodies. Eleven of 14 (78%) of the patients with alloantibodies had also autoantibodies, whereas autoantibodies were found in 16 of 104 (15%) of patients with thalassemia without autoantibodies. The rate of alloantibody formation dropped from 10.1% to 1.7% after application of a strict Rh and Kell matching policy.
A policy of Rhesus and Kell matching without occasional exceptions greatly reduced the development of new alloantibodies and autoantibodies. Self-sufficiency through regular blood donation is necessary for the full implementation of an extended match policy and the prevention of antibody formation in our patients.
重型地中海贫血是阿尔巴尼亚常见的血红蛋白病。然而，关于依赖输血的阿尔巴尼亚地中海贫血患者中红细胞同种免疫和自身免疫的发生率，目前尚无相关数据。
在5年时间里，对118例接受定期输血的地中海贫血患者进行了抗体筛查研究。在此期间，他们仅输注ABO、Rh和Kell系统匹配的血液。这些患者此前因血液短缺曾输注过不匹配的血液。
118例患者中有14例（11.8%）产生了同种抗体。研究开始时已有12例（10.1%）存在同种抗体。在实施严格的Rh和Kell血型匹配政策后，仅2例（1.7%）产生了同种抗体。最常见的抗体是抗-K，其次是抗-D、抗-C、抗-E、抗-c、抗-e、抗-Jk(b)和抗-C(w)。3例患者产生了抗-D加抗-C。5例中有2例抗-K与Rh抗体同时存在。3例中有2例抗-c与抗-E同时存在。大多数抗体（10/14）属于Rh血型系统。除抗-Jk(b)和抗-C(w)外，所有抗体在随访开始时就已存在。在我们的随访期间，27例患者（22.8%）产生了自身抗体。发现同种抗体和自身抗体的存在之间有很强的相关性。14例有同种抗体的患者中有11例（78%）也有自身抗体，而在104例无自身抗体的地中海贫血患者中有16例（15%）发现了自身抗体。实施严格的Rh和Kell血型匹配政策后，同种抗体形成率从10.1%降至1.7%。
严格的Rh和Kell血型匹配政策，无一例外，极大地减少了新的同种抗体和自身抗体的产生。通过定期献血实现自给自足，对于全面实施扩展匹配政策和预防我们患者体内抗体形成是必要的。
