Francesco Cinetto, Italy
Presenter of 1 Presentation
PREVALENCE OF MALIGNANCIES IN CVID PATIENTS WITH INTERSTITIAL LUNG DISEASE (ILD): A SINGLE CENTER RETROSPECTIVE STUDY
Abstract
Background and Aims
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.
Methods
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.
Results
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).
Conclusions
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.