{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T03:33:18Z","timestamp":1791343998741,"version":"4.3.1"},"reference-count":101,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2016,5,17]],"date-time":"2016-05-17T00:00:00Z","timestamp":1463443200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["CTSA Grant Number KL2 TR000136"],"award-info":[{"award-number":["CTSA Grant Number KL2 TR000136"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2016,6]]},"abstract":"<jats:p>Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disorder characterized by overlapping features of myelodysplastic syndromes and myeloproliferative neoplasms. Diagnosis is based on the presence of persistent (&gt;3\u00a0months) peripheral blood monocytosis (&gt;1\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L), along with bone marrow dysplasia. Clonal cytogenetic abnormalities occur in \u223c20\u201330% of patients, while &gt;90% have gene mutations. Mutations involving <jats:italic>TET2<\/jats:italic> (\u223c60%), <jats:italic>SRSF2<\/jats:italic> (\u223c50%), <jats:italic>ASXL1<\/jats:italic> (\u223c40%), and <jats:italic>RAS<\/jats:italic> (\u223c30%) are frequent; with only <jats:italic>ASXL1<\/jats:italic> mutations negatively impacting overall survival. Two molecularly integrated, CMML\u2010specific prognostic models include; the Groupe Fran\u00e7ais des My\u00e9lodysplasies (GFM) and the Molecular Mayo Model (MMM). The GFM model segregates patients into 3 groups based on: age &gt;65\u00a0years, WBC &gt;15\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L, anemia, platelets &lt;100\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L, and <jats:italic>ASXL1<\/jats:italic> mutation status, with respective median survivals of 56 (low), 27.4 (intermediate), and 9.2 (high) months. The MMM is based on <jats:italic>ASXL1<\/jats:italic> mutational status, absolute monocyte count &gt;10\u00a0\u00d7\u00a010<jats:sup>9<\/jats:sup>\/L, hemoglobin &lt;10\u00a0g\/dL, platelets &lt;100\u00a0\u00d7\u00a0109\/L and circulating immature myeloid cells. This model stratifies patients into four groups; high (\u22653 risk factors), intermediate\u20102 (2 risk factors), intermediate\u20101 (1 risk factor) and low (no risk factors), with median survivals of 16, 31, 59, and 97\u00a0months, respectively. Hypomethylating agents such as 5\u2010azacitidine and decitabine are commonly used, with overall response rates of \u223c30\u201340% and complete remission rates of \u223c7\u201317%. Allogeneic stem cell transplant is the only potentially curative option, but is associated with significant morbidity and mortality. Individualized therapy, including epigenetic modifiers and small molecule inhibitors, are exciting prospects. Am. J. 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