Lymphoproliferative Disease in CVID: a Report of Types and Frequencies from a US Patient Registry.
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.
['Yakaboski|Elizabeth|E|', 'Fuleihan|Ramsay L|RL|', 'Sullivan|Kathleen E|KE|', 'Cunningham-Rundles|Charlotte|C|', 'Feuille|Elizabeth|E|']
D000328:Adult D017074:Common Variable Immunodeficiency D005260:Female D006801:Humans D015994:Incidence D000072281:Lymphadenopathy D008218:Lymphocytosis D008223:Lymphoma D008232:Lymphoproliferative Disorders D008297:Male D008875:Middle Aged D015995:Prevalence D019310:Pseudolymphoma D012042:Registries D014481:United States
lymphoproliferative disease CVID common variable immunodeficiency lymphoma lymphoproliferation lymphoproliferative disorder