İki Pediatrik Beta Talasemi Hastasında Transfüzyon İlişkili İmmün Hemolitik Anemi

β-talasemi major hastaları yaşam boyu kan transfüzyonlarına ihtiyaç duyarlar. Ancak, bu hastalarda çoklu allojenik kan transfüzyonlarından dolayı eritrositlere karşı otoantikor ve alloantikor geliştirme riski artmıştır. Burada, son transfüzyonlarından kısa süre sonra otoimmün hemolitik anemi gelişen iki β-talasemi hastasının klinik özellikleri ve tedavi yaklaşımları sunulmuştur. Başlangıçta hastalar immünglobulin ve prednizolon tedavilerine iyi yanıt vermişken, kısa bir süre sonra ilk hastada otoimmün hemolitik anemi tekrarladı. Ancak takiplerinde kotikosteroid tedavisi ile bu hastanın da otoimmün hemolitik anemisi düzeldi. Sonuç olarak talasemi hastalarında allo ve otoantikor oluşumunu azaltmak için kan transfüzyonlarının Rh ve Kell uyumlu olarak yapılması ve eritrosit süspansiyonlarının depolanmadan önce filtrelenmesi önerilmektedir. Anahtar kelimeler: β-Talasemi; Otoimmün Hemolitik Anemi; Çocukluk Çağı.

Anahtar Kelimeler:

β-Talasemi; Otoimmün Hemolitik Anemi; Çocukluk Çağı.

Transfusion-Related Immune Hemolytic Anemia in Two Children with β-Thalassemia

Patients with β-thalassemia major require long-term blood transfusions. However, these patients are at increasing risk of developing anti-red blood cell autoantibodies and alloantibodies due to multiple allogenic blood transfusions. Here, we report the clinical features and management of two patients with β-thalassemia with autoimmune hemolytic anemia followed shortly after last blood transfusions. At the beginning, the patients responded well to the immunoglobulin and prednisolone treatments, but after a short period of time, first patient developed recurrent autoimmune hemolytic anemia. At the end, this patient remained well in the follow up period with corticosteroid treatment. As a result, it is recommended that thalassemia patients should receive blood that matches Rh and Kell antigens, and prestorage leukodepleted erythrocyte suspensions to reduce allo and autoantibody formation. Key words: β-Thalassemia; Autoimmune Hemolytic Anemia; Childhood.

Keywords:

β-Thalassemia; Autoimmune Hemolytic Anemia; Childhood.,

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