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.

Keywords:

• Lymphoma
• lymphoproliferative disorders
• primary immunodeficiency
• PID
• EBV

Acknowledgements

The authors thank The ESID Registry Database (esid.org), The US Lymphoid Malignancy Statistics and The French Lymphopath Network.

Disclosure statement

The authors declare that they have no competing interests.

Availability of data and material

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Additional information

Funding

This study was supported by grants from EU-funded (ERDF) project INTERREG V “RARENET”.