Diagnosis and Risk Stratification in Waldenström Macroglobulinemia.

IF 14.8 2区 医学 Q1 ONCOLOGY Journal of the National Comprehensive Cancer Network Pub Date : 2024-09-01 DOI:10.6004/jnccn.2024.7024
Saurabh Zanwar, Prashant Kapoor
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Abstract

Waldenström macroglobulinemia (WM) is a B-cell lymphoma characterized by the presence of bone marrow lymphoplasmacytic infiltration and circulating monoclonal immunoglobulin M protein. The clinical presentation of WM is variable, ranging from gradually progressive cytopenias, organomegaly, fatigue, B symptoms, and peripheral neuropathy to the more emergent presentation with symptomatic hyperviscosity, cryoglobulinemia, hemolytic anemia-associated symptoms, acquired von Willebrand disease or acquired hemophilia-associated bleeding. Approximately 1 in 5 patients with WM are asymptomatic at diagnosis and classified as having smoldering WM, not requiring WM-directed therapy. Although WM typically has an indolent, relapsing-remitting course, the outcomes are heterogeneous. The prognosis of patients with WM is known to be impacted by certain clinical and laboratory features at initial presentation, with advanced age, elevated serum lactate dehydrogenase, and low serum albumin unfavorably affecting the outcome. Although complications such as histologic transformation or light and/or heavy chain (AL/ALH) amyloidosis are infrequent, their occurrence adversely influences the disease course. The International Prognostic Staging System for WM (IPSS-WM) is a validated model, often used in clinical practice, but needs to be reexamined in the current era. The discovery of the recurrent MYD88L265P gain-of-function point mutation and the subclonal CXCR4 mutations has helped improve our understanding of the WM biology, and the prognostic impact of these mutations is under evaluation, with somewhat inconsistent findings thus far across studies. This review discusses the clinical presentation, diagnostic criteria, and prognostic markers of WM.

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瓦尔登斯特伦巨球蛋白血症的诊断和风险分层。
瓦尔登斯特伦巨球蛋白血症(WM)是一种B细胞淋巴瘤,其特点是存在骨髓淋巴浆细胞浸润和循环中的单克隆免疫球蛋白M蛋白。WM 的临床表现多种多样,既有逐渐进展的细胞减少、器官肿大、乏力、B 症状和周围神经病变,也有症状性高粘度、低温球蛋白血症、溶血性贫血相关症状、获得性冯-威廉氏病或获得性血友病相关出血等急症表现。大约五分之一的 WM 患者在确诊时无症状,被归类为烟雾型 WM,不需要 WM 定向治疗。虽然 WM 的病程通常比较缓慢、复发-缓解,但预后却不尽相同。众所周知,WM 患者的预后会受到初诊时某些临床和实验室特征的影响,高龄、血清乳酸脱氢酶升高和血清白蛋白过低会对预后产生不利影响。虽然组织学转化或轻链和/或重链(AL/ALH)淀粉样变性等并发症并不常见,但其发生会对病程产生不利影响。WM的国际预后分期系统(IPSS-WM)是一个经过验证的模型,常用于临床实践,但在当今时代需要重新审视。复发性 MYD88L265P 功能增益点突变和亚克隆 CXCR4 突变的发现有助于提高我们对 WM 生物学的认识,而这些突变对预后的影响正在评估之中,迄今为止各项研究的结果并不一致。本综述将讨论 WM 的临床表现、诊断标准和预后指标。
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来源期刊
CiteScore
20.20
自引率
0.00%
发文量
388
审稿时长
4-8 weeks
期刊介绍: JNCCN—Journal of the National Comprehensive Cancer Network is a peer-reviewed medical journal read by over 25,000 oncologists and cancer care professionals nationwide. This indexed publication delivers the latest insights into best clinical practices, oncology health services research, and translational medicine. Notably, JNCCN provides updates on the NCCN Clinical Practice Guidelines in Oncology® (NCCN Guidelines®), review articles elaborating on guideline recommendations, health services research, and case reports that spotlight molecular insights in patient care. Guided by its vision, JNCCN seeks to advance the mission of NCCN by serving as the primary resource for information on NCCN Guidelines®, innovation in translational medicine, and scientific studies related to oncology health services research. This encompasses quality care and value, bioethics, comparative and cost effectiveness, public policy, and interventional research on supportive care and survivorship. JNCCN boasts indexing by prominent databases such as MEDLINE/PubMed, Chemical Abstracts, Embase, EmCare, and Scopus, reinforcing its standing as a reputable source for comprehensive information in the field of oncology.
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