İki Pediatrik Beta Talasemi Hastasında Transfüzyon İlişkili İmmün Hemolitik Anemi

Year 2014, Volume: 21 Issue: 1, 52 - 54, 01.02.2014

Arzu Akyay

Abstract

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.

Keywords

β-Talasemi; Otoimmün Hemolitik Anemi; Çocukluk Çağı.

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

Abstract

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.

References

• Singer ST, Wu V, Mignacca R, Kuypers FA, Morel P, Vichinsky EP. Alloimmunization and erythrocyte autoimmunization in transfusion-dependent thalassemia patients of predominantly Asian descent. Blood 2000;96:3369-73.
• Ameen R, Al-Shemmari A, Al-Humood S, Chowdhury RI, Al- Eyaadi O, Al-Bashir A. RBC alloimmunization and autoimmunization among transfusion dependent Arab thalassemia patients. Transfusion 2003;43:1604-10.
• Grainger JD, Makar Y, McManus A, Wynn R. Refractory hyper haemolysis in a patient with β-thalasemia major. Transfus Med 2001;11:55-7.
• Gupta V, Shukla J, Bhatia BD. Autoimmune haemolytic anemia. Indian J Pediatr 2008;75:451-4.
• Kaussi A, Economou M, Gompakis N, Athanassiou-Metaxa M, Pliaki P. Transfusion-related immune hemolytic anemia in thalassemic patients: report of 2 cases. J Pediatr Hematol Oncol 2006;28:402.
• Xu LH, Fang JP, Weng WJ, Huang K, Zhang YT. Autoimmune hemolytic anemia in patients with β- thalassemia major. Pediatr Hematol Oncol 2012;29:235-40.
• Kruatrachuoe M, Sirisinha S, Pacharee P, Chandarayingyong D, Wasi P. An association between thalassemia and autoimmune haemolytic anemia (AIHA). Scan J Hematol 1980;25:259-63.
• de la Rubia J, Moscardo F, Arriaga F, Monteagudo E, Carreras C, Marty ML. Acute parvovirus B19 infection as a cause of autoimmune hemolytic anemia. Haematologica 2000;85:995-7.
• Dhouib N,Guedhami H, Mellouli F, Ben Khaled M, Kaabi H, Belhassen E, et al. Mycoplasma pneumoniae associated with severe autoimmune hemolytic anemia in a child with homozygous beta-thalassemia. Tunis Med 2011;89:652-3.
• Fluit CR, Kunst VA, Drenthe-Schonk AM. Incidence of red cell antibodies after multiple blood transfusion. Transfusion199;30:532-5.
• Segel GB. Hemolytic anemias resulting from extracellular factors. In: Behrman RE, Kliegman RM, Jenson HB,eds. Nelson Textbook of Pediatrics. 18th edition. Philadelphia: WB Saunders; 2007. p. 2042-4.
• Jubinsky PT, Rashid N. Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. Pediatr Blood Cancer 2005;45:347–50.
• Sirchia G, Zanella A, Parraviccini A, Morelati F, Rebulla P, Masera G. Red cell alloantibodies in thalassemia major: results of an Italian cooperative study. Transfusion 1985;25:110-2.
• Grady RW, Akbar AN, Giardina PJ, Hilgartner MW, de Sousa M. Disproportionate lymphoid cell subsets in thalasemia major: the relative contributions of transfusion and splenectomy. Br J Haematol 1985;59:713-24.
• Sokol RJ, Hewitt S, Stamps BK. Autoimmune haemolysis: an 18- year study of 865 cases referred to a regional transfusion centre. Br Med J 1981;282:2023–27.