Table 1.
Scoring system of WAS: −/(+) absent or mild, (+) mild, transient eczema or mild transient infections not resulting in sequelae, + persistent, but therapy responsive eczema and recurrent infections requiring antibiotics and often intravenous immunoglobulin prophylaxis, ++ eczema that is difficult to control and severe threatening infections
| IXLT | XLT | Classic | WAS | XLN | |||
|---|---|---|---|---|---|---|---|
| <1 | 1 | 2 | 3 | 4 | 5 | 0 | |
| Thrombocytopenia | −/+ | + | + | + | + | + | − |
| Small platelets | − | + | + | + | + | + | − |
| Eczema | − | − | (+) | + | ++ | (+)/+/++ | − |
| Immunodeficiency | − | −/(+) | (+) | + | + | (+)/+ | − |
| Infections | − | − | (+) | + | +/++ | (+)/+/++ | − |
| Autoimmunity and/or malignancy | − | − | − | − | − | + | − |
| Congenital neutropenia | − | − | − | − | − | − | + |
1
Because patients with XLT may develop autoimmune disorders or lymphoma, albeit at a lower rate than those with Classic WAS, progression from a score of 1 or 2 to a 5 is possible for XLT.