MALIGNANT LYMPHOMAS IN COMMON VARIABLE IMMUNODEFICIENCY: DATA FROM ITALIAN REFERRAL CARE CENTRES ON SURVEILLANCE, INCIDENCE, RISK-FACTORS AND SURVIVAL.
- Federica Pulvirenti, Italy
Abstract
Background and Aims
An increased prevalence of lymphoma has been observed in common variable immunodeficiency (CVID), the most frequently symptomatic primary immunodeficiency. Herein, we analyzed data on the prevalence of hematological, cancer risk, mortality and survival rate in a cohort of 455 Italian adult CVID patients compared to normative population. Detailed data on CVID patients diagnosed with lymphoma, histology, outcome and possible associated risk-factors were reported.
Methods
Lymphoma incidence rates were evaluated in 455 subjects with CVID enrolled in PID care centres in Rome, Padua and Naples from 1993 to 2017 and compared with incidence rates in AIRTUM database.
Results
CVID patients showed an increased incidence for non-Hodgkin lymphoma (NHL) (Obs=33, SIR=14.3; 95%CI=8.4–22.6), and Hodgkin's lymphoma (HD) (Obs=5, SIR=12.5, 95%CI=3.4-22.4) (Fig.1).The age at lymphoma diagnosis was 32.8 ± 4.6 years for HD and 52.4 ± 13.1 years for NHL. CVID-associated lymphomas were more likely to be of B-cell origin and occurred at extra-nodal sites in 30% of cases reported. T-cell lymphomas (peripheral T-cell lymphoma, angio-immunoblastic T-cell lymphoma and anaplastic T-cell lymphoma) and one primitive effusion cavity lymphomas were also observed. Patients with lymphoma were more likely to have lymphopenia (lymphocytes < 1,000 cell/mm3, OR=3.0, 95%CI=1.1–8.3, p=0.030) and polyclonal lymphocytic infiltration (OR=2.7, 95%CI=1.2–6.3, p=0.016) before cancer diagnosis.CVID patients with lymphoma had a worse survival in comparison to cancer-free CVID (HR: 4.2, 95%CI: 2.8–44.4, Fig.2). Lymphoma was the tird cause of death, following gastric cancer and respiratory infections,
Conclusions
Based on this large series, we highlighted the need of a strategy for hematological malignancy surveillance based on epidemiological studies.