Classic Kaposi sarcoma in 3 unrelated Turkish children born to consanguineous kindreds.
Infection by human herpesvirus 8 (HHV-8) in childhood is common in the Mediterranean basin; however, classic Kaposi sarcoma (KS) is exceedingly rare in children not infected with HIV and not receiving immunosuppression, with only 30 cases having been reported since 1960. We recently reported 2 children with autosomal and X-linked recessive primary immunodeficiencies underlying KS in a context of multiple clinical manifestations. These reports suggested that classic KS in otherwise healthy children might also result from inborn errors of immunity more specific to HHV-8. In this article, we describe 3 unrelated Turkish children with classic KS born to first-cousin parents. The first patient, a girl, developed KS at 2 years of age with disseminated cutaneous and mucosal lesions. The clinical course progressed rapidly, and the patient died within 3 months despite treatment with vincristine. The other 2 children developed a milder form of KS at the age of 9 years, with multiple cutaneous lesions. A boy treated with interferon alpha therapy for 12 months is now in full remission at the age of 14, 2 years after treatment. The second girl is currently stabilized with etoposide, which was begun 4 months ago. None of the 3 children had any relevant familial history or other clinical features. The occurrence of classic KS in 3 unrelated Turkish children, each born to consanguineous parents, strongly suggests that autosomal recessive predisposition may drive the rare occurrence of HHV-8-associated classic KS in children.
Sahin
Gürses
G
The Rockefeller University, 1230 York Avenue, New York, NY 10065, USA.
Palanduz
Ayse
A
Aydogan
Gonul
G
Cassar
Olivier
O
Ertem
A Ulya
AU
Telhan
Leyla
L
Canpolat
Nur
N
Jouanguy
Emmanuelle
E
Picard
Capucine
C
Gessain
Antoine
A
Abel
Laurent
L
Casanova
Jean-Laurent
JL
Plancoulaine
Sabine
S
D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D012514:Sarcoma, Kaposi / Q000235:genetics*; D012878:Skin Neoplasms / Q000235:genetics*; D014421:Turkey