Neşe YARALI, Arzu Duygu DÖNMEZ, Bahattin TUNÇ, Ülker ERTAN, Fulya G. DEMİRÇEKEN

Akut Hemolitik Kriz ve Fulminan Karaciğer Yetmezliği ile Başvuran Wilson Hastalığı Olgusu

Wilson hastalığı çeşitli dokularda bakırın birikmesi ile karakterize bir metabolik bozukluktur. Hastaların çoğunda kronik karaciğer hastalığı veya nörolojik tutuluma bağlı semptomlar görülür. Nadiren, ilk bulgu olarak hemolitik kriz ile ortaya çıkabilir ve acil müdahale edilmediğinde ölümcül sonuçlar doğurabilir. Bu olgu, Coombs negatif hemolitik anemi ayırıcı tanısında Wilson hastalığına dikkat çekmek amacı ile takdim edilmiştir.

Anahtar Kelimeler:

Wilson hastalığı, hemolitik, anemi

A CASE OF WILSON’S DISEASE ACCOMPANYING WITH ACUTE HEMOLYTIC CRISIS AND FULMINANT HEPATIC FAILURE

Wilson’s disease is a metabolic disorder characterized by accumulation of copper in several tissues. Most patients present with manifestations of chronic liver disease or neurologic complications. Rarely, hemolytic crisis may be the first manifestation and this condition may be mortal if not treated urgently. This case is reported to draw attention for Wilson’s disease in the differential diagnosis of Coombs negative hemolytic anemia

Keywords:

Wilson’s disease, hemolytic, crisis,

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