Hemophagocytic Lympho Histiocytosis (HLH)

Author(s): Nijwm Mahilary, Rajib Ray, Rameswar Prasad Mishra, Raj Kumar Paul*

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper-inflammatory condition caused by a highly stimulated, but ineffective immune response, characterized by a dys-regulated activation and proliferation of macrophages leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes and their hematopoietic precursors throughout the reticulo-endothelial system. HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver, or lymph nodes. HLH also occurs as a complication of rheumatic diseases and of malignancies. Awareness of the clinical symptoms and of diagnostic criteria for HLH (Histiocyte society; HLH study 2004) is crucial to starting life-saving therapy in time. We have reported a case of 7-year-old male child presenting with respiratory distress, distension of abdomen and deep jaundice. This case report may be an eye opener to the pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest.

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