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Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds.

Abstract : 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.
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Submitted on : Monday, March 1, 2010 - 11:38:03 AM
Last modification on : Wednesday, August 19, 2020 - 11:16:58 AM

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Gürses Sahin, Ayse Palanduz, Gonul Aydogan, Olivier Cassar, A. Ulya Ertem, et al.. Classic Kaposi Sarcoma in 3 Unrelated Turkish Children Born to Consanguineous Kindreds.. Pediatrics, American Academy of Pediatrics, 2010, 125 (3), pp.e704-e708. ⟨10.1542/peds.2009-2224⟩. ⟨pasteur-00460460⟩

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