Which granulomatous and lymphoid diseases are associated with common variable immunodeficiency (CVID)?

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

Atypical lymphoid hyperplasia due to clonal expansion of B or T lymphocytes has been reported in as many as one third of patients with common variable immunodeficiency. Extranodal sites, such as the lungs, lymph nodes, GI tract, skin, spleen, liver, and parotid gland, may be involved by these lymphoproliferative processes. Lymph nodes show a reactive follicular hyperplasia, atypical hyperplasia, or granulomatous inflammation. Nodular lymphoid hyperplasia in the GI tract with clonal rearrangement of the Ig heavy chain gene or clonal T-cell receptor (TCR) gene rearrangement has been described in otherwise benign cases of lymphoid proliferation in patients with common variable immunodeficiency.

Granulomas have been reported in approximately 8-22% of patients with common variable immunodeficiency. These patients were more likely to have deficient T-cell proliferation to mitogens and antigens. Previous studies have reported 22 common variable immunodeficiency patients with sarcoidosis. Granulomas are indistinguishable from those of classic sarcoidosis and are found in the lungs, liver, spleen, and conjunctivae. Others reported involvement of lymph nodes, skin, GI tract, kidney, bone marrow, or brain. These patients were more likely to have increased frequencies of infections, hepatosplenomegaly, iridocyclitis, autoimmune hemolytic anemia, or immune thrombocytopenic purpura. Successful treatment of granulomas with tumor necrosis factor-alpha antagonists has been reported in a few patients with common variable immunodeficiency.

One report described a possible role of human herpesvirus 8 (HHV-8) in the lymphoproliferative disorders in patients with common variable immunodeficiency. Authors reported high copy numbers of HHV-8 genome in peripheral blood mononuclear cells as well as in the lymph node in patients with common variable immunodeficiency who have lymphoproliferative disorders.


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