In the last decade, CVID patients increased their life expectancy due to improvements in prevention and treatment of severe infections. The relevance of non-infectious complications like ILDs and malignancies in terms of morbidity and mortality is thus increasing.
In this retrospective single-centre study, we analysed a single-center cohort of CVID patients, focusing on the presence of GLILD and malignancies in their clinical history. We analysed cancer prevalence in patients with and without features of ILD.
Among a cohort of 117 patients with diagnosis of CVID, 18 patients had clinical-radiologic and/or histologic features of GLILD (15.4%). The median follow-up was 15 years. In the whole cohort, 28 patients (23.9%) presented a history of malignancy. As expected, these were mainly lymphoproliferative diseases (n=12), (T-Large granular lymphocytic leukemia, B-cell lymphomas) and gastric cancers (n=5). 6/27 patients developed more than one malignancy.Of note, 7 of 18 patients with GLILD features (39%) presented at least one cancer (2 presented T-LGLL and NHL, 1 had pancreatic carcinoma) whilst 21 of the other 99 CVID patients (21%) presented a diagnosis of malignancy; difference was not statistically significant. 6 GLILD patients (33%) presented 8 neoplastic lymphoproliferative diseases (LPDs), while 6 non-GLILD patients presented 6 LPDs (6%); difference was statistically significant (p<0.01).
GLILD patients presented a higher cancer prevalence if compared to the other CVID patients, with a significant increase in LPDs. A more careful follow-up is thus needed in presence of GLILD, since LPDs might be difficult to recognize when occurring in this context.