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Leuk Lymphoma. 2019 Oct 3:1-11. doi: 10.1080/10428194.2019.1672056. [Epub ahead of print]

Primary immunodeficiencies and lymphoma: a systematic review of literature.

Author information

1
Department of Clinical Immunology and Internal Medicine, National Reference Center for Systemic Autoimmune Diseases (RESO), Tertiary Center for Primary Immunodeficiency, Strasbourg University Hospital , Strasbourg , France.
2
Department of Rheumatology, National Reference Center for Systemic Autoimmune Diseases (RESO), Strasbourg University Hospital , Strasbourg , France.
3
Department of Onco-Hematology, Strasbourg University Hospital , Strasbourg , France.
4
UFR Médecine, Université de Strasbourg , Strasbourg , France.
5
Faculty of Dentistry, Strasbourg University - Reference Center for Oral Rare Diseases (O-Rares), Strasbourg University Hospital , Strasbourg , France.
6
INSERM UMR - S1109, Faculté de Médecine, Fédération Hospitalo-Universitaire OMICARE, Fédération de Médecine Translationnelle de Strasbourg (FMTS), Strasbourg University , Strasbourg , France.
7
UFR Sciences Pharmaceutiques, Université de Strasbourg , Strasbourg , France.

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.

KEYWORDS:

EBV; Lymphoma; PID; lymphoproliferative disorders; primary immunodeficiency

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