Article
Familial hemophagocytic lymphohistiocytosis: an autopsy study.
Pediatric pathology & molecular medicine - 1 Jan 2000
Kakkar Nandita, Vasishta R K, Banerjee A K, Marwaha R K, Thapa B R
Abstract excerpt
We describe four classical cases of familial hemophagocytic lymphohistiocytosis (FHL), a macrophage-related, autosomal recessive fatal disorder. Parental consanguinity was present in three cases. All patients presented with fever, neurological involvement of varying degrees, hepatosplenomegaly, cytopenias, deranged liver function tests, and coagulogram, hypofibrinogenemia (three cases), and hyperlipidemia (one...
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