Service d'Hématologie et Thérapie Cellulaire, CHU and Inserm, Poitiers, France.
Laboratoire d'Anatomie Pathologie, CHU, Poitiers, France.
Leuk Lymphoma. 2021 Nov;62(11):2665-2670. doi: 10.1080/10428194.2021.1907379. Epub 2021 Jun 4.
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.
原发性或继发性免疫缺陷（ID）是发生华氏巨球蛋白血症（WM）的一个风险因素，尽管这种情况很少见。我们旨在更好地了解这种情况在现实生活中的发生率以及两种疾病的结局。我们对普瓦图-夏朗德地区登记处的 194 例 WM 进行了回顾性研究，发现 7 例（3.6%）有 ID 病史。在这 7 例 ID 相关 WM 中，有 4 例进展为活动性 WM 疾病，需要针对 WM 进行治疗，WM 诊断与首次治疗之间的中位时间为 1.5 年（范围 0-3 年）。从 ID 到 WM 发病的中位时间为 8 年（1-18 年）。WM 可能源自 ID，尽管这是一种罕见事件。我们的首要行动是通过长期 ID 控制来系统性地降低免疫抑制。尽管 ID 存在，但一半的惰性 WM 仍保持惰性，而对于其余 WM，没有出现 WM 不良风险的情况。
