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. Author manuscript; available in PMC: 2016 Oct 19.
Published in final edited form as: Pediatr Blood Cancer. 2015 Oct 15;63(3):392–397. doi: 10.1002/pbc.25779

TABLE II.

Genetic Predisposition to Pediatric Kaposi Sarcoma

Gene Type Inheritance PID Onset age Presentation Clinical Outcome
WAS Classic XR Wiskott-Aldrich syndrome 14 months Aggressive disseminated cutaneous and systemic KS Complete remission
IFNGR1 Classic AR IFN-γRI deficiency 10 years Aggressive disseminated cutaneous and systemic KS KS progression and death
STIM1 Classic AR STIM1 deficiency 2 years Aggressive disseminated cutaneous and systemic KS KS progression and death
TNFRSF4 Classic AR OX40 deficiency 14 years Aggressive disseminated cutaneous and systemic KS Complete remission

Abbreviations: KS, Kaposi sarcoma; XR, X-linked recessive; AR, autosomal recessive; PID, pediatric immunodeficiency