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Clinical and epidemiologic studies of familial hemophagocytic lymphohistiocytosis in Japan

1 May 1998

Abstract excerpt

BACKGROUND AND PROCEDURE: The etiology of familial hemophagocytic lymphohistiocytosis (FHL), which is characterized by fever, hepatosplenomegaly, pancytopenia, and coagulopathy, remains unknown. We analyzed 43 FHL patients, all with affected siblings, in 18 families who were identified during the period 1986-1995 in Japan. RESULTS: The presence of consanguinity was evident in two families (11%). The majority of...

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PMID: 9544223
DOI: 10.1002/(sici)1096-911x(199805)30:5<276::aid-mpo3>3.0.co;2-c

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