title: Familial clustering of classic Kaposi sarcoma.
abstract: It is widely accepted that there is a causal association between Kaposi sarcoma-associated herpesvirus (KSHV) and Kaposi sarcoma (KS). However, the majority of individuals infected with KSHV never develop KS. Here, we present a unique familial case of classic KS, in which the disease occurs in 4 siblings who have no recognized underlying immunodeficiency. We examine risk factors that could play a role in this condition, including KSHV infection, KSHV DNA load, genetic variants of KSHV, infection with additional viruses, interleukin-6-promoter polymorphism, and HLA genotype. We hypothesize that a genetic susceptibility to KS, in combination with KSHV infection, may play an important role in the presented familial case.
authors.affiliation: Department of Dermatology, Rambam Medical Center, and Department of Cell Biology and Anatomy, Bruce Rappaport Faculty of Medicine, Haifa, Israel.
authors.forename: Emma
authors.initials: E
authors.lastname: Guttman-Yassky
authors.forename: Adina
authors.initials: A
authors.lastname: Cohen
authors.forename: Zippi
authors.initials: Z
authors.lastname: Kra-Oz
authors.forename: Rachel
authors.initials: R
authors.lastname: Friedman-Birnbaum
authors.forename: Elli
authors.initials: E
authors.lastname: Sprecher
authors.forename: Neli
authors.initials: N
authors.lastname: Zaltzman
authors.forename: Eitan
authors.initials: E
authors.lastname: Friedman
authors.forename: Michael
authors.initials: M
authors.lastname: Silbermann
authors.forename: Dina
authors.initials: D
authors.lastname: Rubin
authors.forename: Shai
authors.initials: S
authors.lastname: Linn
authors.forename: Dennis
authors.initials: D
authors.lastname: Whitby
authors.forename: Osnat
authors.initials: O
authors.lastname: Gideoni
authors.forename: Shimon
authors.initials: S
authors.lastname: Pollack
authors.forename: Reuven
authors.initials: R
authors.lastname: Bergman
authors.forename: Ronit
authors.initials: R
authors.lastname: Sarid
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000914:Antibodies, Viral / Q000097:blood; D004279:DNA, Viral / Q000097:blood; D005260:Female; D020022:Genetic Predisposition to Disease; D019288:Herpesvirus 8, Human; D015395:Histocompatibility Antigens Class I / Q000235:genetics / Q000276:immunology; D000949:Histocompatibility Antigens Class II / Q000235:genetics / Q000276:immunology; D006801:Humans; D015850:Interleukin-6 / Q000235:genetics / Q000276:immunology; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011110:Polymorphism, Genetic / Q000276:immunology; D011401:Promoter Regions, Genetic / Q000276:immunology; D012514:Sarcoma, Kaposi / Q000235:genetics* / Q000276:immunology / Q000821:virology
chemical_list: D000914:Antibodies, Viral; D004279:DNA, Viral; D015395:Histocompatibility Antigens Class I; D000949:Histocompatibility Antigens Class II; D015850:Interleukin-6