Chronic myelomonocytic leukemia

Definition:

Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder associated with peripheral blood monocytosis, with an inherent risk for leukemic transformation. CMML has overlapping features of myelodysplastic syndromes (MDS) [DS:H01481] and myeloproliferative neoplasms (MPN). Gene mutations are seen in >90% of patients, with common abnormalities involving; epigenetic regulators (TET2 -60% and ASXL1 -40%), spliceosome components (SRSF2 -50%), transcription factors (RUNX1 -15%) and cell signaling (RAS -30%, CBL -15%).

References:

[1]. Andreas Reiter, et al. Molecular basis of myelodysplastic/myeloproliferative neoplasms. Haematologica. 2009 Dec;94(12):1634-8. [Content Brief]

[2]. M M Patnaik, et al. Cytogenetic and molecular abnormalities in chronic myelomonocytic leukemia. Blood Cancer J. 2016 Feb 5;6(2):e393. [Content Brief]

[3]. Mrinal M Patnaik, et al. Chronic myelomonocytic leukemia: 2016 update on diagnosis, risk stratification, and management. Am J Hematol. 2016 Jun;91(6):631-42. [Content Brief]

[4]. Mrinal M Patnaik, et al. Chronic myelomonocytic leukemia: 2018 update on diagnosis, risk stratification and management. Am J Hematol. 2018 Jun;93(6):824-840. [Content Brief]

[5]. Sanam Loghavi, et al. Recent Updates on Chronic Myelomonocytic Leukemia. Curr Hematol Malig Rep. 2018 Dec;13(6):446-454. [Content Brief]

[6]. Swapna Thota, et al. Myelodysplastic and myeloproliferative neoplasms: updates on the overlap syndromes. Leuk Lymphoma. 2018 Apr;59(4):803-812. [Content Brief]

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