Hospital Universitario y Politécnico La Fe, Valencia.
CIBERONC, Instituto Salud Carlos III, Madrid, Spain.
Curr Opin Oncol. 2018 Nov;30(6):409-417. doi: 10.1097/CCO.0000000000000486.
The present review focuses on the current management of patients with chronic myelomonocytic leukemia (CMML) and the most recent developments in the field.
CMML is a heterogeneous malignant myeloid disorder sharing features of myelodysplastic syndromes (MDS) and myeloproliferative neoplasms and characterized by peripheral blood monocytosis and increased risk of progression to acute leukemia. Its natural course is highly variable and use of CMML-specific prognostic scoring systems is strongly recommended for tailoring treatment. Multiple recent studies have showed that somatic mutations, which are almost always present have a relevant and independent impact on survival but lack a clear role in predicting the response to currently available drugs.
The incorporation of somatic mutations to prognostic scoring systems has improved the prediction of patients' outcomes. Current treatment for CMML remains unsatisfactory. Allogeneic hematopoietic cell transplantation is the only curative option but is applicable to a minority of patients. Usually higher-risk patients displaying MDS-like characteristics are treated with hypomethylating agents (HMAs), whereas those with myeloproliferative features generally receive hydroxyurea or HMAs but none of these drugs substantially modify the natural history of CMML. Newer therapies are clearly needed.
本综述重点关注慢性髓单核细胞白血病(CMML)患者的当前治疗方法和该领域的最新进展。
CMML 是一种异质性恶性髓系疾病,具有骨髓增生异常综合征(MDS)和骨髓增殖性肿瘤的特征,表现为外周血单核细胞增多和向急性白血病进展的风险增加。其自然病程高度可变,强烈建议使用 CMML 特异性预后评分系统来调整治疗。最近的多项研究表明,几乎总是存在的体细胞突变对生存有相关且独立的影响,但在预测现有药物的反应方面尚无明确作用。
体细胞突变的纳入提高了对患者预后的预测能力。CMML 的当前治疗仍不尽如人意。异基因造血细胞移植是唯一的治愈方法,但仅适用于少数患者。通常具有 MDS 样特征的高危患者接受低甲基化剂(HMAs)治疗,而具有骨髓增殖性特征的患者通常接受羟基脲或 HMAs 治疗,但这些药物均不能显著改变 CMML 的自然病程。显然需要新的治疗方法。