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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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.
AbstractList 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.
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.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.
Author Korganow, Anne-Sophie
Mertz, Philippe
Dieudonné, Yannick
Herber, Mathilde
Guffroy, Aurélien
Gies, Vincent
Jung, Sophie
Guffroy, Blandine
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primary immunodeficiency
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Snippet The management of lymphoma in patients with primary immunodeficiency (PID) is challenging because of its poor prognosis and complex therapeutic approaches. We...
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SubjectTerms 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
Title Primary immunodeficiencies and lymphoma: a systematic review of literature
URI https://www.tandfonline.com/doi/abs/10.1080/10428194.2019.1672056
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