A rare presentation of kikuchi disease as hemophagocytic lymphohistiocytosis

Abstract

This case report deals with a young lady in her late 20’s who presented as a case of pyrexia of unknown origin complicated by the rapid development of encephalitis transaminitis and pancytopenia within a span of 24 h and later succumbed to her illness. Her clinical diagnosis and cause of death were hemophagocytic lymphohistiocytosis (HLH) secondary to neutropenic sepsis; however, an autopsy was recommended to ascertain the exact cause of death. Her autopsy examination revealed numerous necrotic enlarged lymph nodes with elevated lactate dehydrogenase levels in peritoneal fluid. The final diagnosis was multiorgan failure due to HLH in the case of Kikuchi disease. Kikuchi disease is usually a benign self-limiting illness presenting most commonly as cervical or axillary lymphadenopathy. This case report highlights a fatal case of Kikuchi disease due to HLH, which is an extremely rare entity.