Table 1.
CVID | ALPS | CAEBV | XMEN | PASLI | CHAI/LATAIE | |
---|---|---|---|---|---|---|
Sinopulmonary infections | Yes | Yes, sometimes with bronchiectasis | Yes, if hypogamm | |||
Ig defects | Yes | High IgM | Often hypogamm | |||
Lymphoproliferative disease | Yes | Yes | Yes | |||
Viremia | Variable | No | EBV | EBV | Often CMV and/or EBV | |
EBV+ B cell lymphoma risk | No | No | Yes | Yes | Yes | |
Lymphocyte tissue infiltration | No | No | No | No |
Yes Mucosal nodules, sometimes obstructive |
Tissue infiltration |
Expanded cell population | Variable | CD3+CD4/8−(B220+) | CD3+CD8+ | CD3+CD8+ (low CD4:CD8) CD8+NKG2D− |
CD3+CD8+ CD3+CCR7− CD8+CD57+ CD20+CD10+ |
More memory T cells and CD21lo B cells |
Disease gene product | Variable | FAS, FASL, caspase 10, N-Ras | Variable | MAGT1 | p110δ or p85α | CTLA4 or LRBA |