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Familial erythrophagocytic lymphohistiocytosis. report of two cases and clinicopathologic review
Author(s) -
Perry Michael C.,
Harrison Edgar G.,
Burgert E. Omer,
Gilchrist Gerald S.
Publication year - 1976
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197607)38:1<209::aid-cncr2820380132>3.0.co;2-w
Subject(s) - medicine , hepatosplenomegaly , pancytopenia , pediatrics , sepsis , hemophagocytic lymphohistiocytosis , meningitis , surgery , pathology , disease , bone marrow
An unusual syndrome of hepatosplenomegaly and fever followed by rapid deterioration and death has been described in 38 children from 21 families Pancytopenia, liver dysfunction, and bleeding developed prior to death from hemorrhage, sepsis, or lymphocytic meningitis. This report reviews the literature and adds a set of twins to the reported cases.