Kaan DEMİRÖREN, Ümran ÇALIŞKAN, Saadet DEMİRÖREN

Evans sendromu: İki olgu sunumu

Amaç: Evans sendromu, otoimmun trombositopeni (ITP) ve otoimmun hemolitik aneminin (OİHA) birlikte veya peşi sıra gelişimi ile karakterizedir. Tedaviye rağmen hastaların çoğu kronik ya da tekrarlamalar gösteren bir sürece girmekte olup önemli derecede mortaliteye sahiptir. Çocukluk çağında nadir görüldüğü için bu olguları sunmak istedik. Olgu sunumu: Sunumuzda biri iki aylık, diğeri bir yaşında iki kız hasta anlatılmaktadır. Her iki hastada da ileri derecede anemi dışında, trombositopeniye ait klinik bir bulgu yoktu. Anemi, trombositopeni, direkt Coombs pozitifliği, retikülositoz birlikteliği ve ikincil nedenlerin olmadığının gösterilmesi ile hastalarda Evans sendromu düşünüldü. Her iki hastamıza da başlanan yüksek doz prednizolon tedavisine rağmen direkt Coombs pozitifliği ve retikülositoz düzelmedi. Yalnızca ikinci olgunun trombosit sayısında kalıcı artış sağlandı. İntravenöz immunoglobulin tedavisi de ek bir yarar sağlamadı. Sonuç: Evans sendromu İTP veya hemolitik anemi şeklinde başlayabileceği için akut İTP tanısı düşünülen her hastada direkt Coombs ile retikülosit sayısına bakılmalıdır.

Anahtar Kelimeler:

Prednizolon, Trombositopeni, Anemi, hemolitik, otoimmün, Çocuk, Süt çocuğu, İlaç tedavisi

Evans syndrome: Two cases report

Objective: Evans syndrome is defined as the simultaneous or sequential occurrence of autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Despite the therapy, most cases with Evans syndrome have a chronic and relapsing course. Mortality rate is considerably high. Since Evans syndrome is rarely seen in childhood, we aimed to report these cases. Case report: We presented two cases, one of which was a 2-months-old girl, and the other was a 1-year-old girl, both have severe anemia as a major finding, and have no clinical evidence of thrombocytopenia. Our diagnosis was Evans syndrome because of the presence of anemia, thrombocytopenia, positive direct Coombs test, reticulocytosis, and the absence of secondary causes. Although we treated them with high dose prednisolone, neither direct Coombs test nor reticulocytosis returned to normal. Only, one year old girl had a permanent rise in thrombocyte count. Intravenous immune globulin therapy did not provide an additional advantage. Conclusion: Since Evans syndrome can present itself as ITP or AIHA, direct Coombs test and reticulocyte count should be studied routinely for those patients.

Keywords:

Prednisolone, Thrombocytopenia, Anemia, Hemolytic, Autoimmune, Child, Infant, Drug Therapy,

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