Premium
Familial occurrence of histiocytosis
Author(s) -
Frisell E.,
Björkstén B.,
Holmgren G.,
ÅNgström T.
Publication year - 1977
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1977.tb01295.x
Subject(s) - malignant histiocytosis , consanguinity , histiocytosis , malignancy , disease , medicine , genetics , pathology , biology , pediatrics , histiocyte
The clinical and histological findings of four children with lethal histiocytosis are reported. The children belonged to two sibships and originated from a Swedish geographical isolate. Consanguinity between the parents was established for one of the sibships, belonging to a pedigree in which malignant disease occurred in two generations. The observations indicate that the type of histiocytosis investigated is caused by homozygosity for a single recessive gene. The association between histiocytosis and malignancy is discussed.