title: Familial occurrence of histiocytosis.
abstract: 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.
mesh_terms: D003241:Consanguinity; D005260:Female; D005808:Genes, Recessive; D006646:Histiocytosis, Langerhans-Cell / Q000235:genetics*; D006801:Humans; D007223:Infant; D008297:Male; D010375:Pedigree; D013548:Sweden