Abstract

Haemophagocytic lymphohostiocytosis (HLH) is a life threatening but benign condition of severe hyper inflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. The hallmark of the disease is low or absent NK cells and CD8+ T lymphocytes cytotoxicity. There are two forms of syndrome –Familial hemophagocytic lymphohistiocytosis (FHL) of infants is a rare autosomal recessive disorder demonstrating mutations in perforin gene in 30-50% cases and other reactive hemophagocytic syndrome (RHS) at any age. Epstein-Barr virus, the pathogen that most commonly triggers infection associated HPS (IA-HPS). HLH is fatal if left untreated. We report a case of HLH in an 8 year old male child who presented in paediatric outdoor with complains of high grade fever (>103⁰c), pancytopenia, hepatosplenomegaly along with rashes over face and scalp. Biochemical investigations show very high serum ferritin level i.e. 2830µg/l, hypertriglyceridaemia and hypofibrinogenamia. Bone Marrow aspirate and biopsy examination revealed all hematopoietic components with prominence of histiocytes and evidence of hemophagocytosis.

Keywords: Hemophagocytic lymphohistiocytosis, Reactive.