Article
Familial and acquired hemophagocytic lymphohistiocytosis.
European journal of pediatrics - 1 Feb 2007
Janka Gritta E
Abstract excerpt
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled proliferation of activated lymphocytes and histiocytes secreting high amounts of inflammatory cytokines. Cardinal signs and symptoms are prolonged fever, hepatosplenomegaly and pancytopenia. Characteristic biochemical markers include elevated triglycerides, ferritin and low fibrinogen....
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