Hemophagocytic Lymphohistiocytosis Associated with Visceral Leishmaniasis: Varied Presentation

Visceral leishmaniasis (VL) is endemic in many parts of India. Rarely, it may be complicated by hemophagocytic lymphohistiocytosis (HLH) that has varied presentation and course. We describe two cases of VL complicated by HLH that were markedly different in clinical presentation, course and management. First case presented with Fever of unknown origin whereas second case had fever with severe bleeding manifestations. VL was diagnosed by bone marrow aspiration and serum rk39 immunodiagnostic test respectively in these cases. HLH was diagnosed by HLH 2004 diagnostic criteria. VL was treated by intravenous amphotericin B in both cases. HLH was managed by treating primary disease in the first case whereas steroid was given for management in the second case. High index of suspicion is crucial for early diagnosis of HLH to reduce morbidity and mortality.