关键词: Waldenström's macroglobulinemia acquired von Willebrand disease hyperviscosity syndrome plasma exchange

Mesh : Humans Waldenstrom Macroglobulinemia / therapy complications Male Female Retrospective Studies Aged Plasmapheresis / methods Middle Aged Blood Viscosity Immunoglobulin M / blood Treatment Outcome Syndrome Rituximab / therapeutic use Aged, 80 and over

来  源:   DOI:10.1111/1744-9987.14094

Abstract:
BACKGROUND: Hyperviscosity syndrome (HVS) is a significant complication in Waldenström\'s macroglobulinemia (WM), although the significance of plasmapheresis in clinical practice has not been clarified. To confirm the efficacy and safety of plasmapheresis followed by primary therapy for WM with HVS, we retrospectively conducted this study.
METHODS: Untreated WM with HVS, or serum immunoglobulin M (IgM) levels ≥4000 mg/dL, were analyzed. The basic therapeutic flow was conducted as follows: (1) performing plasmapheresis, (2) followed by primary therapy without rituximab, and (3) performing the second cycle. The serum IgM reduction rate and adverse events (AEs) were evaluated.
RESULTS: Ten patients were enrolled. The median serum IgM levels at diagnosis, post-plasmapheresis, after the first cycle of primary therapy, and after the second cycle were 5174, 2900, 3286, and 2657 mg/dL, respectively. No patients experienced IgM flare or bleeding AEs.
CONCLUSIONS: The therapeutic flow offers sufficient efficacy and safety in WM with HVS.
摘要:
背景:高黏度综合征(HVS)是Waldenström巨球蛋白血症(WM)的重要并发症,尽管血浆置换在临床实践中的意义尚未明确。为了证实血浆置换后的主要治疗用HVS治疗WM的疗效和安全性,我们回顾性地进行了这项研究.
方法:未经HVS处理的WM,或血清免疫球蛋白M(IgM)水平≥4000mg/dL,进行了分析。基本治疗流程如下:(1)进行血浆置换,(2)随后是没有利妥昔单抗的主要治疗,以及(3)执行第二循环。评估血清IgM降低率和不良事件(AEs)。
结果:纳入10例患者。诊断时的血清IgM水平中位数,血浆置换后,在第一周期的初级治疗之后,第二个周期后分别为5174、2900、3286和2657mg/dL,分别。没有患者出现IgM耀斑或出血AE。
结论:治疗流程在合并HVS的WM中提供了足够的疗效和安全性。
公众号