Division of Malignant Hematology, Moffitt Cancer Center, Tampa, FL, USA.
Division of Malignant Hematology, Moffitt Cancer Center, Tampa, FL, USA.
Best Pract Res Clin Haematol. 2020 Jun;33(2):101131. doi: 10.1016/j.beha.2019.101131. Epub 2019 Dec 6.
Many prognostic scoring systems have been developed for chronic myelomonocytic leukemia (CMML). Although these efforts have been informative, no single model has been considered the consensus for CMML prognostication and all models are only moderately prognostic. CMML clinical models utilize mainly hematology and morphology parameters to estimate risk. A better understanding of cytogenetics and the genomic landscape of CMML have resulted in integrated risk models such as CMML Prognostic Scoring System (CPSS)-Mol and Mayo Molecular that may provide better prognostic accuracy for an individual patient. For example, frameshift/nonsense ASXL1 mutations have been consistently shown to confer inferior outcomes leading to its incorporation into some of the major risk classification systems. Prognostication in the setting of therapeutic interventions such as hypomethylating agents and allogeneic hematopoietic cell transplantation have also garnered considerable interest. Despite having many validated risk models available, not a single system is universally adopted. Herein, we will provide an overview of how these systems evolved and progress toward a uniform system.
许多预后评分系统已被开发用于慢性髓单核细胞白血病(CMML)。尽管这些努力提供了有价值的信息,但没有一个单一的模型被认为是 CMML 预后的共识,而且所有模型的预后能力都只是中等。CMML 临床模型主要利用血液学和形态学参数来估计风险。对细胞遗传学和 CMML 的基因组景观的更好理解导致了综合风险模型的出现,例如 CMML 预后评分系统(CPSS)-Mol 和 Mayo 分子,这些模型可能为个体患者提供更好的预后准确性。例如,移码/无义 ASXL1 突变一直被证明会导致预后不良,因此被纳入一些主要的风险分类系统中。在接受去甲基化药物和异基因造血细胞移植等治疗干预的情况下进行预后评估也引起了相当大的兴趣。尽管有许多经过验证的风险模型可用,但没有一个系统被普遍采用。在此,我们将概述这些系统的发展情况以及向统一系统迈进的进展。