{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T12:25:30Z","timestamp":1793363130175,"version":"4.5.0"},"reference-count":11,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,12,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Myelodysplastic syndrome (MDS)\/myeloproliferative neoplasm (MPN) overlap syndromes are unique myeloid neoplasms, with overlapping features of MDS and MPN. They consist of four adult onset entities including chronic myelomonocytic leukemia (CMML), MDS\/MPN-ring sideroblasts-thrombocytosis (MDS\/MPN-RS-T), BCR-ABL1 negative atypical chronic myeloid leukemia (aCML) and MDS\/MPN-unclassifiable (MDS\/MPN-U); with juvenile myelomonocytic leukemia (JMML) being the only pediatric onset entity. Among these overlap neoplasms, CMML is the most frequent and is hallmarked by the presence of sustained peripheral blood monocytosis with recurrent mutations involving TET2 (60%), SRSF2 (50%) and ASXL1 (40%); with RAS pathway mutations and JAK2V617F being relatively enriched in proliferative CMML subtypes (WBC \u226513 \u00d7 109\/L). CMML usually presents in the 7th decade of life, with a male preponderance and is associated with a median overall survival of &amp;lt;36 months. Adverse prognosticators in CMML include increasing age, high WBC, presence of circulating immature myeloid cells, anemia, thrombocytopenia and truncating ASXL1 mutations. While allogeneic stem cell transplantation remains the only curative option, given the late onset of this neoplasm and high frequency of comorbidities, most patients remain ineligible. Hypomethylating agents such as azacitidine, decitabine and oral decitabine\/cedazuridine have been US FDA approved for the management of CMML, with overall response rates of 40-50% and complete remission rates of &amp;lt;20%. While these agents epigenetically restore hematopoiesis in a subset of responding patients, they do not impact mutational allele burdens and eventual disease progression to AML remains inevitable. Newer treatment modalities exploiting epigenetic, signaling and splicing abnormalities commonly seen in CMML are much needed.<\/jats:p>","DOI":"10.1182\/hematology.2020000163","type":"journal-article","created":{"date-parts":[[2020,12,5]],"date-time":"2020-12-05T04:23:41Z","timestamp":1607142221000},"page":"460-464","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":45,"title":["Evidence-Based Minireview: Myelodysplastic syndrome\/myeloproliferative neoplasm overlap syndromes: a focused review"],"prefix":"10.1182","volume":"2020","author":[{"given":"Mrinal M.","family":"Patnaik","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terra","family":"Lasho","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","reference":[{"issue":"20","key":"2020121621261231100_B1","doi-asserted-by":"crossref","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","article-title":"The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia [published correction appears in Blood. 2016;128(3):462-463]","volume":"127","author":"Arber","year":"2016","journal-title":"Blood"},{"key":"2020121621261231100_B2","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.canep.2016.03.011","article-title":"Myeloid malignancies in the real-world: occurrence, progression and survival in the UK\u2019s population-based Haematological Malignancy Research Network 2004-15","volume":"42","author":"Roman","year":"2016","journal-title":"Cancer Epidemiol"},{"issue":"1","key":"2020121621261231100_B3","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1002\/ajh.25684","article-title":"Chronic myelomonocytic leukemia: 2020 update on diagnosis, risk stratification and management","volume":"95","author":"Patnaik","year":"2020","journal-title":"Am J Hematol"},{"issue":"5","key":"2020121621261231100_B4","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1038\/s41375-019-0690-7","article-title":"Clinical, molecular, and prognostic correlates of number, type, and functional localization of TET2 mutations in chronic myelomonocytic leukemia (CMML)-a study of 1084 patients","volume":"34","author":"Coltro","year":"2020","journal-title":"Leukemia"},{"issue":"4","key":"2020121621261231100_B5","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1038\/leu.2015.337","article-title":"Age-related mutations and chronic myelomonocytic leukemia","volume":"30","author":"Mason","year":"2016","journal-title":"Leukemia"},{"issue":"11","key":"2020121621261231100_B6","doi-asserted-by":"crossref","first-page":"2206","DOI":"10.1038\/leu.2014.125","article-title":"ASXL1 and SETBP1 mutations and their prognostic contribution in chronic myelomonocytic leukemia: a two-center study of 466 patients","volume":"28","author":"Patnaik","year":"2014","journal-title":"Leukemia"},{"issue":"5","key":"2020121621261231100_B7","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1016\/j.bbmt.2017.01.078","article-title":"Allogeneic hematopoietic cell transplantation for adult chronic myelomonocytic leukemia","volume":"23","author":"Liu","year":"2017","journal-title":"Biol Blood Marrow Transplant"},{"issue":"1","key":"2020121621261231100_B8","doi-asserted-by":"crossref","first-page":"e270","DOI":"10.1038\/bcj.2014.90","article-title":"Chronic myelomonocytic leukemia in younger patients: molecular and cytogenetic predictors of survival and treatment outcome [published correction appears in Blood Cancer J. 2015;5(2):e280]","volume":"5","author":"Patnaik","year":"2015","journal-title":"Blood Cancer J"},{"issue":"7","key":"2020121621261231100_B9","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1002\/ajh.25488","article-title":"Suboptimal response rates to hypomethylating agent therapy in chronic myelomonocytic leukemia; 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