Hemophagocytic lymphohistiocytosis presenting with acute liver failure and central nervous system involvement in early infancy

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal and likely underdiagnosed disease characterized by unregulated histiocyte proliferation, hypercytokinemia and hemophagocytosis, causing life-threatening tissue damage and organ failure. We report a case of a 56-day-old infant presenting with fever, acute liver failure, and neurological manifestations as presenting features that succumbed to rapidly progressive HLH. Our objective is to emphasize the importance of early diagnosis by high suspicion in varied initial presentation of HLH so that life-saving therapy may be instituted in time.

Keywords

• Acute liver failure
• familial hemophagocytosis lymphohistiocytosis
• infant
• neurological manifestations