Murat YILMAZ, Sibel TAMER, Tahir Kurtuluş YOLDAŞ, Bülent GÜVEN

Nadir Bir Serebrovasküler Olay Nedeni; Hemofagositik Sendromun Gözlendiği Still Hastalığı

Amaç: Erişkin Still hastalığı etyoloji ve patogenezi bilinmeyen sistemik inflamatuvar bir hastalıktır. Tanı klinik ve laboratuvar bulgularının kombinasyonu ile olası diğer hastalıkların dışlanmasına dayanır. Hemofagositik sendrom ateş, hepatosplenomegali, hipofibrinojenemi, hipertrigliseridemi, sitopeni, kemik iliği, dalak veya lenf nodlarında hemofagositoz ile karakterize bir sendromdur. Erişkin Still hastalığı bu sendroma neden olan sistemik romatolojik hastalıklardan biriymiş gibi görünmektedir. Bu yazıda, erişkin Still hastalığında gelişen ve kortikosteroit tedavisine yanıt veren hemofagositik sendrom ve serebellar hemoraji olgusu sunulmuştur. Hastalığın klinik ve laboratuar bulguları ile ayırıcı tanısı ve tedavisi tartışılmıştır. Bu iki hastalığın birlikteliğinde serebrovasküler olay gelişebileceği göz ardı edilmemelidir.

A Rare Cause of Cerebrovascular Event; Hemophagocytic Syndrome Observed in Still’s Disease

Adult Still’s disease is a systemic inflammatory disorder of unknown etiology and patogenesis. Diagnosis is based on the combination of clinical and laboratory findings and exclusion of the other probable diseases. Hemophagocytic syndrome is a syndrome characterized by fever, hepatosplenomegaly, hypofibrinogenemia, hypertriglyceridemia, and cytopenia, with hemophagocytosis in bone marrow, spleen or lymph nodes. Adult Still’s disease seems to be one of systemic rheumatological diseases that may cause this syndrome. In this article a case of hemofagositik syndrom with cerebellar hemorrage who developped in adult Still’s disease and responded to cortocosteroid treatment has been presented. The clinical and laboratory finding , differential diagnosis and the treatment of the case is discussed. It is suggested that the possibility of cerebrovascular event development should not be overlooked when this two entities are together.

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