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 |