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. Author manuscript; available in PMC: 2017 Dec 19.
Published in final edited form as: Annu Rev Immunol. 2015 Dec 23;34:121–149. doi: 10.1146/annurev-immunol-041015-055620

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