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Chronic Myelomonocytic Leukemia (CMML)

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Abstract

The term chronic “myelomonocytic” leukemia (CMML) indicates that all cells of the myeloid lineage are involved (myelo-), but emphasizes the prominence of monocytoid features (“-mono-”). The hallmarks of CMML are peripheral monocytosis >1, 000/μl, with <20% bone marrow blasts and the presence of bone marrowdysplasia.CMML shares clinical and biological features with both myelodysplastic syndromes (MDS) and chronic myeloproliferative diseases (CMPDs), and may take on predominantly myelodysplastic (MD-CMML) or myelprolifearative (MP-CMML) characteristics (e.g., Ref. [1]). There is a dynamic evolution through increasing monocyte counts in approximately one-third of patients (see Fig. 7.1). MDSRA patients may develop peripheral monocytosis during the course of their disease and ultimately progress to CMML [2]. Approximately one-fifth of patients with MDS present with a monocyte count of above 10% in the peripheral blood without fulfilling the FAB/WHO criteria for the diagnosis of CMML. A high incidence of disease progression to CMML has been reported for this subgroup [3] (see Fig. 7.1).

Static and dynamic classification of CMML (adapted from Ref. [4]). Approximately 20% of patients with MDS present with a monocyte count >10%, but do not fulfill the WHO criteria for the diagnosis of CMML. 1/3 of these patients progresses to MD-CMML and 1/3 of these may go on to progress to MP-CMML. RA Refractory anemia; RARS refractory anemia with ringed sideroblasts; CMML chronic myelomonocytic leukemia; PB peripheral blood

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Pleyer, L., Neureiter, D., Faber, V., Greil, R. (2010). Chronic Myelomonocytic Leukemia (CMML). In: Greil, R., Pleyer, L., Faber, V., Neureiter, D. (eds) Chronic Myeloid Neoplasias and Clonal Overlap Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-79892-8_7

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