Table 1.

Potential predictors of hematological malignancy risk in patients with common variable immune deficiency (CVID).

Pre-existing or concomitant lymphoproliferative disease (2.5-fold increased risk of lymphoma; p=0.005) (25)

Longstanding lymphadenopathy or splenomegaly (16, 19, 26)

Autoimmune manifestations, including cytopenia, in some (16, 18, 19, 25) but not all (25) studies

Non-infectious gastrointestinal involvement/enteropathy in some (16, 19) but not all (25) studies

High IgM levels at diagnosis of CVID in some studies (16, 26, 35); low IgM in other studies (19)

Greater age at CVID diagnosis (25)

Female sex (35); Male sex (19)

Late-onset combined immunodeficiency (loCID) phenotype (36)

Mutations in CTLA4, BACH2, TNFSR13B, PIK3CD, PIK3R1, CD27, CD70, NFkB, CD19, TWEAK, CD21, ICOS, IRF2BP2 (37, 38)

EBV susceptibility (39)