Primary immunodeficiencies and lymphoma: a systematic review of literature. Issue 2 (28th January 2020)

• Record Type: Journal Article
• Authors: Herber, Mathilde; Mertz, Philippe; Dieudonné, Yannick; Guffroy, Blandine; Jung, Sophie; Gies, Vincent; Korganow, Anne-Sophie; Guffroy, Aurélien
• Is Part Of: Leukemia & lymphoma. Volume 61:Issue 2(2020)
• Page Start: 274
• Page End: 284
• Publication Date: 2020-01-28
• Subjects: Lymphoma -- lymphoproliferative disorders -- primary immunodeficiency -- PID -- EBV; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2019.1672056
• Languages: English
• ISSNs: 1042-8194

Abstract

Abstract: The management of lymphoma in patients with primary immunodeficiency (PID) is challenging because of its poor prognosis and complex therapeutic approaches. We conducted a systematic literature review of case-reports, case-series, and cohorts indexed in MEDLINE reporting the association of lymphoma and PID. One hundred and eighty-two articles were selected out of 787. We identified 386 cases. Median age at diagnosis of PID and lymphoma was 9.5 and 12 years old, respectively. T-cell deficiencies were the main PIDs associated with lymphoma (57%). The most prevalent lymphoma was diffuse large B-cell lymphoma (33.5%). Epstein–Barr Virus-driven lymphomas were mostly observed in innate immunodeficiencies (when reported). Complete response to treatment was observed in 65.8% of the cases. Death occurred in 38.2%. Few allogenic stem cell transplantations were performed (29 cases). Our detailed analysis of the literature provides a landscape of lymphoma's occurrence in PID. Devoted studies in specific sub-groups of patients at risk are needed to develop dedicated protocols.