Contemporary CMML Risk Stratification and Management.

IF 2.7 3区医学 Q2 HEMATOLOGY

Current Hematologic Malignancy Reports Pub Date : 2025-07-04 DOI:10.1007/s11899-025-00753-2

Dahniel Sastow, Arjun Syal, Douglas Tremblay

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引用次数: 0

Abstract

Purpose of review: Chronic myelomonocytic leukemia (CMML), a rare myelodysplastic/myeloproliferative neoplasm (MDS/MPN) overlap syndrome, poses diagnostic and therapeutic challenges due to its heterogeneity and rarity. This review highlights updates to CMML diagnostic criteria, examines risk stratification models and their clinical implications, and outlines both established and emerging therapies for this rare and likely underrecognized hematologic malignancy.

Recent findings: Updated diagnostic criteria from 2022 reduce the monocyte threshold to 0.5 × 10⁹/L for CMML diagnosis, which will reclassify many cases previously diagnosed as MDS. Risk stratification models continue to be refined allowing for improved prediction and may help select appropriate patients for allogeneic stem cell transplantation, the only curative therapeutic modality in CMML. Although trials in CMML were long relegated to subpopulation of MDS studies, there has recently been a flourishing of novel therapies being tested specifically in CMML. These include lenzilumab (anti-GM-CSF) and IO-202 (anti-LILRB4), which have demonstrated promising early efficacy signals but require further study. Established treatments, which include hypomethylating agents and hydroxyurea as well as the JAK1/2 inhibitor ruxolitinib, provide limited survival benefits in CMML, underscoring the urgent need for novel therapeutic development. Coordinated dedicated research efforts have started to evaluate new agents in CMML. Along with further diagnostic and prognostic refinement, these advances are welcomed for this rare and heterogenous disease.

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当代cml风险分层与管理。

慢性髓细胞白血病（CMML）是一种罕见的骨髓增生异常/骨髓增生性肿瘤（MDS/MPN）重叠综合征，由于其异质性和罕见性，给诊断和治疗带来了挑战。本综述重点介绍了CMML诊断标准的更新，检查了风险分层模型及其临床意义，并概述了针对这种罕见且可能未被充分认识的血液恶性肿瘤的现有和新兴治疗方法。最新发现：2022年更新的诊断标准将CMML诊断的单核细胞阈值降低到0.5 × 10⁹/L，这将重新分类许多先前诊断为MDS的病例。风险分层模型继续完善，以改进预测，并可能有助于选择合适的患者进行同种异体干细胞移植，这是CMML唯一的治愈治疗方式。虽然CMML的试验长期以来一直被降级为MDS研究的亚群，但最近有大量的新疗法正在专门针对CMML进行测试。这些药物包括lenzilumab（抗gm - csf）和IO-202（抗lilrb4），它们已经显示出有希望的早期疗效信号，但需要进一步研究。现有的治疗方法，包括低甲基化药物和羟基脲以及JAK1/2抑制剂ruxolitinib，在CMML中提供有限的生存益处，强调迫切需要开发新的治疗方法。协调专门的研究工作已经开始评估CMML中的新药物。随着进一步的诊断和预后的改进，这些进展对这种罕见和异质性疾病是受欢迎的。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Hematologic Malignancy Reports ONCOLOGY-HEMATOLOGY

CiteScore

6.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： his journal intends to provide clear, insightful, balanced contributions by international experts that review the most important, recently published clinical findings related to the diagnosis, treatment, management, and prevention of hematologic malignancy. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas, such as leukemia, lymphoma, myeloma, and T-cell and other lymphoproliferative malignancies. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research. Commentaries from well-known figures in the field are also provided.