EMİNE ÖZDEMİR, Yasemin Işık BALCI, Havva EVRENGÜL, Gülay SÖNMEZ, Tuğçe BOZKURT, Eda KARADAGLI, Aziz POLAT

Mycophenolate Mofetil Induced Remission in Steroid-Refractory Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia (AIHA) is a disease that is seen in 1/10.000 people and characterized by forming antibodies against red blood cells and degradation of these red blood cells in reticuloendothelial system. Even autoimmune diseases can accompany, it is seen usually idiopathic. Paleness due to anemia, jaundice, tachycardia, darkening in urine color, hepatosplenomegaly are frequently seen clinical findings. Clinically normochrome and normocytic anemia, reticulocytosis, polychromasia in peripheral smear, spherocytes, indirect hyperbilirubinemia and increased lactate dehydrogenase are seen. Direct coombs test is the diagnostic test. Steroids are the first line drugs in treatment. The dosage and the treatment duration is formed according to patient’s clinical situation. The treatment is checked by complete blood count, reticulocyte and Coombs test. Immunosuppressor treatments are given to patients that don’t respond to treatment in 4-6 weeks or less patients who has recurrence at the time of decreasing the treatment dosage of corticosteroids. In few patient, that did not respond steroids, immunosuppressive treatments are used. Here, we present a patient, who is diagnosed with AIHA that we couldn’t manage remission by steroid treatment, and no response to rituxumab as an immunosuppressor, but treated successfully with microphenolat mofetil.

Mikofenolat Mofetil ile Remisyon Sağlanan Steroide Dirençli Otoimmün Hemolitik Anemi

Otoimmün hemolitik anemi (OİHA), eritrosit antijenlerine karşı antikor oluşması, antikorla kaplanmış eritrositlerin retiküloendotelyal sistemde yıkımı ile karakterize, 1/10,000 sıklıkta görülen bir hastalıktır. Otoimmün hastalıklar eşlik edebileceği gibi genelde idiyopatik olarak görülmektedir. Anemiye bağlı solukluk, sarılık, taşikardi, idrar renginde koyulaşma, hepatosplenomegali sık görülen klinik bulgular arasında yer almaktadır. Genellikle normokrom ve normositer anemi, retikülositoz, periferik yaymada polikromozi, sferosit, indirekt hiperbilirubinemi, laktat dehidrogenaz artışı görülmektedir. Direkt Coombs testi tanısal tetkiktir. Tedavide steroidler ilk tercihtir. Tedavinin dozu ve süresi hastanın kliniğine göre düzenlenmektedir. Tam kan sayımı, retikülosit ve Coombs testi ile tedavi takip edilmektedir. Dört-altı haftada yanıt alınamayan hastalarda veya kortikosteroid tedavisinin azaltıldığı dönemde tekrar nüks eden daha az olguda ise immünosüpresör tedaviler uygulanmaktadır. Burada, OİHA tanısı konulan ve steroid tedavisi ile remisyon sağlayamadığımız immünosüpresif tedavilerden rituksimab tedavisine cevap alamadığımız mikofenolat mofetil ile tedavi edilen gören bir olgu sunulmuştur.

PDF

___

Lanzkowsky P. Extracorpuscular Hemolytic Anemia. In: Lanzkowsky P. Manual of Pediatric Hematology and Oncology. 5th ed. USA: Elsevier, 2011: 247-56.

Gehrs BC, Friedberg RC. Autoimmune Hemolytic Anemia. American Journal of Hematology 2002; 69: 258-71.

Sève P, Bourdillon L, Sarrot-Reynauld F. Autoimmune hemolytic anemia and common variable immunodeficiency: a case-control study of 18 patients. Medicine (Baltimore) 2008; 87: 177.

Aladjidi N, Leverger G, Leblanc T. New insights into childhood autoimmune hemolytic anemia: a French national observational study of 265 children. Haematologica 2011; 96: 655.

Liesveld JL, Rowe JM, Lichtman MA. Variability of the erythropoietic response in autoimmune hemolytic anemia: analysis of 109 cases. Blood 1987; 69: 820.

Garraty G. Immune hemolytic anemia associated with drug therapy. Blood Rev 2010; 24: 143-50.

Collins PW, Newland AC. Treatment modalities of autoimmune blood disorders. Semin Hematol 1992; 29: 64-74.

Gobert D, Bussel JB, Cunningham-Rundles C. Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients. Br J Haematol 2011; 155: 498-508.

Zimmer-Molsberger B, Knauf W, Tiel E. Mycophenolate mofetil for severe autoimmune haemolytic anemia. Lancet 1997; 350: 1003-4.

Zaja F, Iacona I, Masolini P. B cell depletion with rituximab as treatment for immune hemolytic anemia and chronic thrombocytopenia. Haematologica 2002; 87: 189-95.

Barros MMO, Blajchman MA, Bordin JO. Warm autoimmune hemolytic anemia: recent progress in understanding the immunobiology and the treatment. Transfus Med Rev 2010; 24: 195-21.