title: Primary haemophagocytic syndrome in a young girl.
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.
authors: Department of Medicine, Calicut Medical College, Calicut 673008, Kerala, India. sasidharanpk@gmail.com
authors: P K
authors: PK
authors: Sasidharan
authors: C
authors: C
authors: Prasanth Varghese
authors: P
authors: P
authors: Sandeep
authors: R
authors: R
authors: Sreejith
authors: Mohammed
authors: M
authors: Shaan
authors: P V
authors: PV
authors: Shiji
authors: H
authors: H
authors: Satish
authors: M
authors: M
authors: Feroz