Primary haemophagocytic syndrome in a young girl.
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Date
2011-01
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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
pancytopenia. 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.
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Sasidharan P K, Varghese Prasanth C, Sandeesp P, Sreejith R, Mohammed Shaan, Shiji P V, Satish H, Feroz M. Primary haemophagocytic syndrome in a young girl. National Medical Journal of India. 2011 Jan-Feb; 24(1): 19-20.