CVID courses with infections, autoimmunity, benign lymphoproliferations, and increased incidence of malignancies such as lymphomas and carcinomas. The most prevalent neoplasm is lymphoma followed by gastric cancer. Our objective was to evaluate the prevalence of malignancies in CVID patients from 1981 to 2019, followed at Division of Clinical Immunology and Allergy, HCFMUSP, São Paulo/Brazil.
Review of medical records of adult CVID patients.
We evaluated 180 CVID patients (55% women). Mean age at onset was 13.2y, mean age at diagnosis, 33.6y. Time between onset and diagnosis was 20.3y and time of IVIG replacement was 21.4y. Thirty-five patients developed cancer: 13 gastric tumor; 6 non-Hodgkin and 1 Hodgkin lymphoma; 7 skin; 2 colorectal; 2 thyroid; 2 breast; 1 gallbladder; 1 adrenal cancer. Average disease time of patients was 36y, and mean age at cancer diagnosis 45.2y, being their mean ID diagnosis before cancer 30.8y. Regarding gastric cancer, 12 patients presented adenocarcinoma and one well differentiated neuroendocrine tumor. Five died, mean time of fatal evolution, 48.6mo. There was no age difference between gastric patients’ survivors and non-survivors. Ten patients presented gastric atrophy, autoimmunity and lymphocyte number alterations, 11 intestinal metaplasia, 7 H. pylori (+) and all had chronic diarrhea and IgA deficiency. We observed an increase in gastric tumors’ prevalence in the last 5 years, being before 2013, 0.23 new cases/year and after, 1.20 new cases/year.
Unlike reported in literature, the most frequent malignancy observed in our cohort was gastric cancer. We observed a striking increase in incidence in the last five years.