Waldenström macroglobulinemia and relationship to immune deficiency

Abstract

Primary or secondary immune deficiency (ID) is a risk factor, although rare, to develop Waldenström macroglobulinemia (WM). We aimed to better understand the incidence of this occurrence in the real-life and the outcome of either entity. We conducted a review of 194 WM in the Poitou-Charentes registry and identified 7 (3.6%) with a prior history of ID. Across the 7 WM with ID, 4 progressed to active WM disease and required treatment for WM with a median time between WM diagnosis and the first treatment of 1.5 years (range 0–3). The median time from ID to WM occurrence was 8 years (1–18). WM could develop from ID, although a rare event. Our first action was to systematically decrease immunosuppression with long-term control of ID. Half of indolent WM remained indolent despite ID and for remaining WM none appeared of poor risk WM.

Keywords:

• Waldenstrom macroglobulinemia
• immune deficiency
• immunosuppression
• relationship
• real-life experience

Acknowledgements

Authors thank CRCs, staff, patients and their families, rheumatologist and nephrologist physicians. We wish to thank Jeffrey Arsham, an American medical translator under contract at the Poitiers CHU, for his review of our original English language document.

Ethical approval

This article does not contain any studies with animals performed by any of the authors. All procedures performed in studies involving human participants were in accordance with the ethical standards of the local IRB of the CHU of Poitiers (CNIL, French data protection authority, number: CHU86-R2018-03-01) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Authors contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Anthony Levy, Xavier Leleu, Cécile Tomowiak. The first draft of the manuscript was written by Anthony Levy, Xavier Leleu, Cécile Tomowiak and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Disclosure statement

The authors declare that they have no conflict of interest.

Data availability statement

Data base declared to the CNIL (French data protection authority).

Code availability

CHU86-R2018-03-01