Primary immunodeficiencies and lymphoma: a systematic review of literature

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 a...

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Published inLeukemia & lymphoma Vol. 61; no. 2; pp. 274 - 284
Main Authors Herber, Mathilde, Mertz, Philippe, Dieudonné, Yannick, Guffroy, Blandine, Jung, Sophie, Gies, Vincent, Korganow, Anne-Sophie, Guffroy, Aurélien
Format Journal Article
LanguageEnglish
Published United States Taylor & Francis 28.01.2020
Subjects
Child
EBV
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - epidemiology
Herpesvirus 4, Human
Human health and pathology
Humans
Immunologic Deficiency Syndromes - diagnosis
Immunologic Deficiency Syndromes - epidemiology
Immunologic Deficiency Syndromes - therapy
Life Sciences
Lymphoma
Lymphoma, Large B-Cell, Diffuse
lymphoproliferative disorders
PID
primary immunodeficiency
Primary Immunodeficiency Diseases
lymphoproliferative disorders
EBV
primary immunodeficiency
PID
Lymphoma
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Summary: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.
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ISSN:1042-8194
1029-2403
1029-2403
DOI:10.1080/10428194.2019.1672056