Primary immunodeficiencies and lymphoma: a systematic review of literature.

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.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Herber M,Mertz P,Dieudonné Y,Guffroy B,Jung S,Gies V,Korganow AS,Guffroy A

doi

10.1080/10428194.2019.1672056

subject

Has Abstract

pub_date

2020-02-01 00:00:00

pages

274-284

issue

2

eissn

1042-8194

issn

1029-2403

journal_volume

61

pub_type

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