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Waldenström macroglobulinemia and relationship to immune deficiency
Anthony Levy1, Stéphanie Guidez1, Céline Debiais2
1Service d'Hématologie et Thérapie Cellulaire, CHU and Inserm, Poitiers, France.
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.
Insights
Individuals with immune deficiency (ID) rarely develop Waldenström macroglobulinemia (WM). This study found that 3.6% of WM patients had a history of ID, with some progressing to active disease.
Area of Science:
- Hematology
- Immunology
Background:
- Primary or secondary immune deficiency (ID) is a rare risk factor for Waldenström macroglobulinemia (WM).
- Understanding the incidence and outcomes of WM in patients with ID is crucial for clinical management.
Purpose of the Study:
- To investigate the incidence of Waldenström macroglobulinemia (WM) in patients with a history of immune deficiency (ID).
- To analyze the clinical outcomes and disease progression in WM patients with co-existing ID.
Main Methods:
- Retrospective review of 194 Waldenström macroglobulinemia (WM) cases from the Poitou-Charentes registry.
- Identification of patients with a prior history of immune deficiency (ID).
- Analysis of time to WM diagnosis, treatment initiation, and disease course.
Main Results:
- Seven out of 194 (3.6%) WM patients had a history of ID.
- Four of these seven patients progressed to active WM requiring treatment.
- The median time from ID diagnosis to WM occurrence was 8 years.
- Half of the indolent WM cases remained indolent despite ID.
Conclusions:
- Waldenström macroglobulinemia (WM) can develop in patients with immune deficiency (ID), though it is a rare occurrence.
- Systematic reduction of immunosuppression and long-term ID control are initial management strategies.
- WM in ID patients can present with indolent or non-poor risk disease characteristics.
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