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

Authors
  • Yakaboski, Elizabeth1
  • Fuleihan, Ramsay L.2
  • Sullivan, Kathleen E.3
  • Cunningham-Rundles, Charlotte4
  • Feuille, Elizabeth5
  • 1 Massachusetts General Hospital, 55 Fruit St, Bulfinch 165, Boston, MA 02114, USA
  • 2 Ann and Robert H Lurie Children’s Hospital of Chicago, Chicago, IL, USA
  • 3 Children’s Hospital of Philadelphia, Philadelphia, PA, USA
  • 4 Icahn School of Medicine at Mount Sinai, New York, NY, USA
  • 5 Weill Cornell Medicine, New York, NY, USA
Type
Published Article
Journal
Journal of Clinical Immunology
Publisher
Springer-Verlag
Publication Date
Mar 17, 2020
Volume
40
Issue
3
Pages
524–530
Identifiers
DOI: 10.1007/s10875-020-00769-8
PMID: 32185577
PMCID: PMC7310568
Source
PubMed Central
Keywords
License
Unknown

Abstract

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.

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