Mathilde Herber, France
Strasbourg University Hospital Clinical ImmunologyPresenter of 1 Presentation
PRIMARY IMMUNODEFICIENCIES AND LYMPHOMA: A SYSTEMATIC REVIEW
Abstract
Background and Aims
Management of lymphoma in patients with PID remains challenging. Prognosis are worse, with difficult therapeutic approaches.
Methods
We conducted a systematic literature review in the medline (Pubmed) of the association of lymphoma and PID (articles reporting one or more original cases). We describe the characteristics, treatment and outcomes of lymphoma occurring in PID and the type of PID concerned.
Results
Our bibliographic research identified 782 articles on Medline of wich 386 single cases with lymphoma (F/M Sex Ratio: 0.7) were retained for analyses. Median age at diagnosis of PID and lymphoma was 9.5 and 12 years, respectively. T-cell deficiencies were the main PID concerned by lymphoma (57%) followed by CVID (21.5%). A previous benign lymphoproliferation and autoimmunity was reported in 15.7% and 18.5 % of cases. The diagnosis of lymphoma was made 2 to 5 year after PID in 89.8% of patients. Most represented lymphomas were DLBCL (33.5%). Lymphomas were diffuse (stage III/IV) (67.2%) and extranodal (76.4%). EBV-driven lymphoma (52.8%) were mostly present in innate deficiencies, represented by DLBCL (38.2%), follicular lymphoma (13.2%) and mixed cellularity classical HL (10.5%). Complete response to treatment was observed in 65.8%. Death occurred in 38.2% and was caused by infections in 32.3%. Few stem cell transplantations were performed (19 cases).
Conclusions
This detailed analysis of individual cases provide a landscape of the type and the course of lymphoma occurring in PID and open the door to dedicate studies in specific sub-group of patients at risk, and dedicated therapeutic protocols. Finally, lymphoma could also revealed PID.