A Fatal Case of Pyrexia of Unknown Origin: Hemophagocytic Lymphohistiocytosis?

ABSTRACT Aim To highlight the need of high suspicion of hemophagocytic lymphohistiocytosis (HLH) in pyrexia of unknown origin (PUO) and also the need of specific guidelines for the management of the disease. Background The HLH is a rare disease of overactive histiocytes and lymphocytes. Although seen in all age groups, it is less common in adults as compared with infants. It usually presents with fever, hepatosplenomegaly, pancytopenia, lymphadenopathy, and rash. Cutaneous involvement is seen in as many as 65% of patients. Case Report A 25-year-old male presented with high-grade fever and cough since 4 weeks. Examination at presentation revealed only hepatosplenomegaly. During the course of the disease, he developed pancytopenia, but there was no lymphadenopathy or neurological or cutaneous manifestations. The patient was thoroughly investigated, but it remained inconclusive. He did not respond to any antibiotics, antimalarials, or antituberculars. There was an initial response to steroids, but it did not sustain. Bone marrow remained normal throughout the course of illness. Based on other features, we made a diagnosis of HLH, but the patient's condition worsened rapidly and proved to be fatal despite all treatment. Conclusion and clinical significance The HLH must be suspected in a case of PUO especially in presence of pancytopenia and hepatosplenomegaly. It is important to look for other features like hyperferritinemia and hypertriglyceridemia to reach an early diagnosis as bone marrow features may present very late during the course of illness. Early diagnosis is all the more important in view of rapidly progressive fatal course of the disease. How to cite this article Bhuta P, Patel A, Yadav L, Patel U, Kosamia V, Muley A. A Fatal Case of Pyrexia of Unknown Origin: Hemophagocytic Lymphohistiocytosis? J Postgrad Med Edu Res 2017;51(4):188-191.