Author(s): Sasidharan PK, Prasanth Varghese C, Sandeep P, Sreejith R, Shaan M, , Sasidharan PK, Prasanth Varghese C, Sandeep P, Sreejith R, Shaan M,
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Abstract Haemophagocytic syndrome is a life-threatening systemic illness characterized by an uncontrolled inflammatory response. Patients present with fever, hepatosplenomegaly, jaundice and liver dysfunction, neurological manifestations and often pancytopenla. Bone marrow, lymph node, hepatic or splenic biopsy shows macrophages with Ingested blood cells or their precursors. Laboratory markers include elevated triglycerides and ferritin, low fibrinogen with normal or low erythrocyte sedimentation rate (ESR). Familial haemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder. Secondary haemophagocytic syndrome results from infections, malignancy and collagen vascular disorders. We describe a young girl with primary haemophagocytic syndrome.
This article was published in Natl Med J India
and referenced in Journal of Thrombosis and Circulation: Open Access