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5TH NATIONAL CONFERENCE OF PEDIATRIC RHEUMATOLOGY, KOLKATA, 29TH & 30TH SEPTEMBER 2007
Subham Bhattacharya*, Soumik Dhar**, Priynkar Pal***, Subroto Chakrabartty****
*MD PGT, **DCH PGT,*** Assistant Professor, **** Professor
Institute of Child Health, Kolkata
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by uncontrolled nonmalignant proliferation of T lymphocytes and macrophages leading to a cytokine storm and often pose diagnostic difficulty to a clinician. Cardinal symptoms are prolonged fever, hepatosplenomegaly and cytopenias. Central nervousHemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by uncontrolled nonmalignant proliferation of T lymphocytes and macrophages leading to a cytokine storm and often pose diagnostic difficulty to a clinician. Cardinal symptoms are prolonged fever, hepatosplenomegaly and cytopenias. Central nervous system (CNS) symptoms are common. Biochemical markers include elevated triglyceride and ferritin, low fibrinogen..
Three patients of Hemophagocytic lymphohistiocytosis aged 3 to 10 years admitted during the period of October 2006 to September 2007 in Institute of Child Health,
Kolkata have been studied retrospectively.
All three children presented with prolonged fever (>15 days), one child having jaundice and ascites, and two having mucosal bleeding. All children were drowsy at presentation with one child having generalised tonic clonic convulsion. There were no specific family history . Mild hepatosplenomegaly was present in all three children.
Complete hemogram revealed pancytopenia in two children with the third having anaemia and thrombocytopenia. D-dimer was raised in all(>10,000). In all three children the diagnosis of Hemophagocytosis was confirmed by bone marrow examination.
All three children were treated by supportive measures and corticosteroid. With treatment two children recovered gradually and one child had a fatal outcome.
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