Zanwar Saurabh, Rajkumar S Vincent
Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Leukemia. 2025 Jul 23. doi: 10.1038/s41375-025-02654-y.
Clonal plasma cell disorders encompass a spectrum of conditions such as multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), Waldenström macroglobulinemia (WM), and immunoglobulin light chain (AL) amyloidosis. In MGUS, SMM, and MM, progression risk varies widely and is influenced by a complex interplay of tumor burden, cytogenetic abnormalities, bone marrow microenvironment, and host factors. Waldenström macroglobulinemia, while usually indolent, presents its own distinct spectrum of molecular abnormalities and disparate clinical outcomes. In AL amyloidosis, clinical trajectories are heavily dictated by the nature and extent of organ involvement. In this review, we provide a comprehensive overview of current risk stratification schema used across the spectrum of clonal plasma cell disorders, highlight the strengths and limitations of major risk stratification frameworks, and provide our recommendations for clinical practice.
克隆性浆细胞疾病包括一系列病症，如多发性骨髓瘤（MM）、意义未明的单克隆丙种球蛋白病（MGUS）、冒烟型多发性骨髓瘤（SMM）、华氏巨球蛋白血症（WM）和免疫球蛋白轻链（AL）淀粉样变性。在MGUS、SMM和MM中，进展风险差异很大，受肿瘤负荷、细胞遗传学异常、骨髓微环境和宿主因素等复杂相互作用的影响。华氏巨球蛋白血症虽然通常进展缓慢，但有其独特的分子异常谱和不同的临床结局。在AL淀粉样变性中，临床病程很大程度上取决于器官受累的性质和程度。在本综述中，我们全面概述了克隆性浆细胞疾病谱中目前使用的风险分层方案，强调了主要风险分层框架的优势和局限性，并为临床实践提供了我们的建议。
