What is the increased risk for malignancy in common variable immunodeficiency (CVID)?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

About 2–8% of patients with common variable immunodeficiency develop non-Hodgkin's lymphoma (NHL). Most of these patients have the B-cell immunophenotype and are frequently negative for Epstein-Barr virus (EBV). Lymphoma is 3 times more common in women with common variable immunodeficiency than in men with common variable immunodeficiency. Malignant lymphomas are most common in the fifth to seventh decade of life and are uncommon in childhood. These malignant lymphomas are usually extranodal and frequently locate in mucosal regions. European registry data revealed a correlation between serum immunoglobulin M (IgM) level at presentation and the eventual development of lymphoid malignancy. In contrast, the IgG level did not predict this phenotype.

Patients with common variable immunodeficiency also have markedly increased risk for gastric carcinoma than general population. Other malignancies include colon cancer, breast cancer, gastric cancer, prostate cancer, ovarian cancer, oral cancer, and melanoma.


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