Lymphoproliferative Disease in CVID: a Report of Types and Frequencies from a US Patient Registry

Elizabeth Yakaboski, Ramsay L. Fuleihan, Kathleen E. Sullivan, Charlotte Cunningham-Rundles, Elizabeth Feuille

Research output: Contribution to journalArticlepeer-review

33 Scopus citations


Purpose: Lymphoproliferative disease in common variable immunodeficiency disease (CVID) is heterogeneous in pathogenesis and ranges from non-malignant lymphoid hyperplasia to lymphoma. Methods: The United States Immunodeficiency Network (USIDNET) patient registry was queried for lymphoproliferative diseases reported in CVID patients. Diagnoses included as possible manifestations of lymphoproliferation included lymphadenopathy, lymphoid hyperplasia, lymphocytic inflammation, lymphocytosis, and gammopathy. Results: Among 1091 CVID patients, lymphoproliferative conditions were reported in 17.2% (N = 188). These conditions included lymphadenopathy (N = 192, 12.3%), lymphoid hyperplasia or lymphocytic inflammation (N = 50, 4.6%), lymphocytosis (N = 3, 0.3%), and gammopathies (N = 3, 0.3%). Of the 188 patients with lymphoproliferative conditions, 15 (8%) also had a diagnosis of lymphoma, while the remaining 173 (92%) did not. Nine (4.8%) had a diagnosis of non-lymphomatous malignancy including basal cell carcinoma (N = 3, 1.6%), thyroid carcinoma (N = 2, 1.1%), gynecologic cancer (N = 2, 1.1%), testicular cancer (N = 1), and vocal cord carcinoma (N = 1). CVID patients with lymphoma were older than patients with lymphoproliferative disease who did not have a diagnosis of lymphoma at the time of analysis (median age 49 vs. 35 years, p = 0.005). CVID patients with lymphoproliferative disease had 2.5 times higher odds of having chronic lung disease compared with those with lymphoma (OR = 0.4, p = 0.049). There were no significant differences in the frequency of autoimmune, gastrointestinal, hepatic, or granulomatous disease between these populations. Conclusions: While CVID patients are at increased risk for lymphoma, lymphoproliferation may be observed in the absence of a concurrent hematologic or solid tumor malignancy.

Original languageEnglish
Pages (from-to)524-530
Number of pages7
JournalJournal of Clinical Immunology
Issue number3
StatePublished - 1 Apr 2020


  • CVID
  • common variable immunodeficiency
  • lymphoma
  • lymphoproliferation
  • lymphoproliferative disease
  • lymphoproliferative disorder


Dive into the research topics of 'Lymphoproliferative Disease in CVID: a Report of Types and Frequencies from a US Patient Registry'. Together they form a unique fingerprint.

Cite this