TABLE II.
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