Michaux J L, Martiat P
Department of Haematology, Cliniques Universitaires St-Luc, Université Catholique de Louvain, Brussels, Belgium.
Leuk Lymphoma. 1993 Jan;9(1-2):35-41. doi: 10.3109/10428199309148501.
Chronic myelomonocytic leukaemia (CMML), a disorder belonging to the group of myelodysplastic syndromes, has a number of peculiar features which raise the question as to whether it should be considered a distinct entity in its own right. The problems associated with its classification and diagnosis are discussed in this report using all currently available tools from clinical data to molecular genetics, including morphology, histology, cellular biology and cytogenetics. Three groups of patients can be identified (isolated monocytosis with a mild degree of dysplasia, severe cytopenia and the most frequent type with proliferative symptoms dominating the clinical picture). The latter group is close to atypical chronic myeloid leukaemia and perhaps these two entities should be regarded as a single one. Classification of the disease is further complicated by the possibility of evolution from one subgroup into another one and by the finding that CMML can also arise as a disorder secondary to other myeloproliferative (MPS) or myelodysplastic (MDS) syndromes. No specific marker of the disease has been identified by cytogenetics or molecular biology. Due to all these facts, we believe that CMML should perhaps be viewed more pragmatically by considering the use of prognostic factors that could at least help to define different groups of patients who may require different therapeutic strategies. We conclude that CMML is a heterogeneous syndrome with features of both MPS and MDS, encompassing primary and secondary stem cell disorders and varying widely in its clinical presentation. This heterogeneity should stimulate the search for reliable predictors of evolution which would allow a better definition of CMML subtypes based on prognostic factors.
慢性粒单核细胞白血病（CMML）属于骨髓增生异常综合征，具有许多独特特征，这引发了它是否应被视为一个独立病种的问题。本报告使用从临床数据到分子遗传学的所有现有工具，包括形态学、组织学、细胞生物学和细胞遗传学，来讨论与其分类和诊断相关的问题。可将患者分为三组（伴有轻度发育异常的孤立性单核细胞增多症、严重血细胞减少症以及临床症状以增殖性症状为主导的最常见类型）。后一组与非典型慢性髓性白血病相近，或许这两个病种应被视为一个整体。疾病的分类因可能从一个亚组演变为另一个亚组以及CMML也可作为继发于其他骨髓增殖性（MPS）或骨髓增生异常（MDS）综合征的疾病而进一步复杂化。细胞遗传学或分子生物学尚未鉴定出该疾病的特异性标志物。基于所有这些事实，我们认为或许应更务实看待CMML，可以考虑使用预后因素，这至少有助于界定可能需要不同治疗策略的不同患者群体。我们得出结论，CMML是一种具有MPS和MDS特征的异质性综合征，涵盖原发性和继发性干细胞疾病，临床表现差异很大。这种异质性应促使人们寻找可靠的病情演变预测指标，从而能够根据预后因素更好地界定CMML亚型。
