Alloimmunization against Rh and Kell blood groups antigens is the main obstacle for blood transfusion in transfusion dependent thalassemia patients in Iran

Abstract is not required for Editorialis not required for Editorial (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 8 No. 6, June 2017. ISSN – [0976-3198] Int J Case Rep Images 2017;8(6):358–363. www.ijcasereportsandimages.com Dorgalaleh et al. 358 CASE REPORT OPEN ACCESS Alloimmunization against Rh and Kell blood groups antigens is the main obstacle for blood transfusion in transfusion dependent thalassemia patients in Iran Akbar Dorgalaleh, Mohammad Saeed Gholami, Mohammad Shokuhiyan, Mohsen Valikhani, Esmaei Saneei Moghaddam, Majid Naderi ALLOIMMUNIZATION IN THALASSEMIA MAJOR Thalassemia is the most common inherited singlegene disorder, causes by decrease or absence of α-globin or β-globin chain production. The disorder commonly inherited in autosomal recessive manner and is more common in areas with high rate of consanguinity [1–3]. Thalassemia belt is an extensive area which extend from Mediterranean east through Middle-East and India to Southeast Asia and south through Africa. Estimated incidence of thalassemia in this area is varies from 1–20% depend on area. Iran as a Middle-East country with high rate of consanguineous marriage has a considerable number of patients with β-thalassemia major [4–6]. The precise incidence of disorder is not clear in Iran but it was estimated that there are between two and three million beta thalassemia carriers and about 20,000 patients with beta thalassemia major. The main therapeutic choice in these patients is packed red blood cell (pRBC) transfusion. Continuous blood transfusion imposed a number of transfusion related complications, most importantly iron overload and related complications as well as alloimmunization against transfused red blood cell antigens [6–10]. The reported rate of alloimmunization Akbar Dorgalaleh1, Mohammad Saeed Gholami1, Mohammad Shokuhiyan1, Mohsen Valikhani1, Esmaei Saneei Moghaddam2, Majid Naderi3 Affiliations: 1Department of Hematology and Blood Transfusion, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran; 2Genetic Research Center in Non-Communicable Disease, Zahedan University of Medical sciences, Zahedan, Iran; 3Iranian Blood Transfusion Organization Research Center, Zahedan, Iran.. Corresponding Author: Akbar Dorgalaleh, Tehran, Iran; Email: dorgalaleha@gmail.com Received: 17 May 2017 Accepted: 18 May 2017 Published: 01 June 2017 among transfused dependent patients with thalassemia varies between 4–50% and has a lower incidence in homologues populations. Some of these alloantibodies are important and even cause severe life-threatening transfusion related hemolytic reactions while others are clinically insignificant. Both of alloantibodies and autoantibodies may decrease survival of transfused pRBC and increase transfusion rate. Such patients may require immunosuppressive drugs, splenectomy as well as other alternative treatments. Therefore, alloimmunization and autoimmunization can significantly affect patients’ quality of life and overall survival [11–13]. ALLOIMMUNIZATION IN THALASSEMIA MAJOR IN IRAN A considerable number of studies were performed in patients with β-thalassemia major in different areas of Iran. The most common used method for detection of alloimmunization, was conventional tube technique (~80%), while gel method was used in minority (~20%) [5–8]. In the majority of studies, in addition to alloantibodies, autoantibody (8 out of 13 studies) (61.5%) also were detected [1, 3, 4, 12]. Among these studies, the rate of autoimmunization ranges from 1–~19% [4, 9]. The rate of alloimmunization varied between ~3–76%. The lower incidence of alloimmunization was reported in Tehran province, while the highest incidence was observed in Isfahan province [6, 9]. Among alloantibodies the majorities are against Rh and Kell blood group systems. The prevalence of alloantibody against, Rh system ranged from 7.5–100% and this prevalence for Kell system varied from 14–60% [3]. Among these studies on Iranian patients, the rate of splenectomy was reported from ~8– 100%. The rate of alloimmunization against these totally splenectomies patients was ~4% (Table 1). In Rh blood group system, most of antibodies directed against, E, C and c antigens respectively, while in Kell blood group system the majority were directed against K antigen (Table 2). EDITO IAL OPEN A CE S