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. 2014 Aug 2;34(6):696–747. doi: 10.1007/s10875-014-0065-9
PATIENTS Dock-8 n = 15(%) Non-Dock8 n = 11(%)
Cutaneous viral infections
HSV 85 40
VZV 16 33
HPV 38 60
MC 33 20
Abscesses 27 10
Mucocutaneous candidiasis 14 20
URTI 100 100
Pneumonia 93 60
Bronchiectasis 35 30
Allergic Symtoms
Eczema 100 (severe) 27 mostly mild, (dermatitis 40%)
Food allergy 70 -
Asthma 30 -
Autoimmunity
Sclerosing cholangitis 20 -
Autoimmune hemolytic anemia - 14
Other autoimmune manifestations - 45%(3 Leukocytoklastic vasculitis, 1 hypothyroidism, and 1 immune thrombocytopenic purpura
Malignancy 27 (2 siblings from 2 families died of lymphoma, 1 developed multiple malignancy (squamous cell Ca, leiomyosarcoma, basal cell Ca A sibling died of lymphoma
Nerological complication 20 1 patient (epilepsy, brain infarct)
Outcome 3 died (one with post -BMT comp., 2 with malignancy All alive