Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria; Ludwig Boltzmann Institute for Hematology & Oncology, Vienna, Austria.
Best Pract Res Clin Haematol. 2020 Jun;33(2):101137. doi: 10.1016/j.beha.2019.101137. Epub 2019 Dec 15.
Chronic myelomonocytic leukemia (CMML) is defined by myelodysplasia, pathologic accumulation of monocytes and a substantial risk to transform to secondary acute myeloid leukemia (sAML). In recent years, minimal diagnostic criteria for classical CMML and CMML-variants were proposed. Moreover, potential pre-stages of CMML and interface conditions have been postulated. Oligomonocytic CMML is a condition where the absolute peripheral blood monocyte count does not reach a diagnostic level but all other criteria for CMML are fulfilled. Among potential pre-stages of CMML, clonal and non-clonal conditions have been described, including idiopathic monocytosis (IMUS) and clonal monocytosis of unknown significance (CMUS). Patients with myelodysplastic syndromes (MDS), clonal cytopenia of unknown significance (CCUS), clonal hematopoiesis of indeterminate potential (CHIP) and idiopathic cytopenia of undetermined significance (ICUS) may also progress to CMML. The current article provides an overview of pre-CMML conditions and oligomonocytic CMML, with special reference to diagnostic criteria, differential diagnoses, clinical outcomes and management.
慢性髓单核细胞白血病（CMML）的特征为骨髓增生异常、单核细胞病理性蓄积，并且向继发性急性髓系白血病（sAML）转化的风险较大。近年来，提出了经典 CMML 和 CMML 变异型的最低诊断标准。此外，还推测了 CMML 的潜在前期阶段和交界条件。寡单细胞 CMML 是指外周血单核细胞绝对计数未达到诊断水平，但符合 CMML 所有其他标准的一种情况。在 CMML 的潜在前期阶段中，已描述了克隆和非克隆情况，包括特发性单核细胞增多症（IMUS）和意义未明的克隆性单核细胞增多症（CMUS）。骨髓增生异常综合征（MDS）、意义未明的克隆性血细胞减少症（CCUS）、不定潜能克隆性造血（CHIP）和特发性血细胞减少症（ICUS）患者也可能进展为 CMML。本文重点介绍了 CMML 的前期状态和寡单细胞 CMML，特别参考了诊断标准、鉴别诊断、临床结果和治疗。
