{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T23:08:31Z","timestamp":1781910511474,"version":"3.54.5"},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2017,4,29]],"date-time":"2017-04-29T00:00:00Z","timestamp":1493424000000},"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","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":[[2017,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Atypical chronic myeloid leukemia (aCML) is an aggressive myeloid neoplasm with overlapping features of myelodysplastic syndromes (prominent granulocytic dysplasia) and myeloproliferative neoplasms (neutrophilic leukocytosis). We studied 25 molecularly\u2010annotated and World Health Organization defined aCML patients; median age 70 years, 84% males. Cytogenetic abnormalities were seen in 36% and gene mutations in 100%. Mutational frequencies were, <jats:italic>ASXL1<\/jats:italic> 28%<jats:italic>, TET2<\/jats:italic> 16%, <jats:italic>NRAS<\/jats:italic> 16%<jats:italic>, SETBP1<\/jats:italic> 12%<jats:italic>, RUNX1<\/jats:italic> 12%, <jats:italic>ETNK1<\/jats:italic> 8%, and <jats:italic>PTPN11<\/jats:italic> 4%. Fifteen patients (60%) had &gt;1 mutation, while 9 (36%) had \u22653. The median overall survival (OS) was 10.8 months and at last follow up (median 11 months), 17 (68%) deaths and 2 (8%) leukemic transformations were documented. On univariate analysis, survival was adversely impacted by advanced age (<jats:italic>P<\/jats:italic>\u2009=\u2009.02), low hemoglobin (<jats:italic>P<\/jats:italic>\u2009=\u2009.01), red blood cell transfusion dependence (<jats:italic>P<\/jats:italic>\u2009=\u2009.03), high white blood cell count (<jats:italic>P<\/jats:italic>\u2009=\u2009.02), <jats:italic>TET2<\/jats:italic> (<jats:italic>P<\/jats:italic>\u2009=\u2009.03), <jats:italic>NRAS<\/jats:italic> (<jats:italic>P<\/jats:italic>\u2009=\u2009.04), <jats:italic>PTPN11<\/jats:italic> (<jats:italic>P<\/jats:italic>\u2009=\u2009.02) mutations and the presence of \u22653 gene mutations (<jats:italic>P<\/jats:italic>\u2009=\u2009.006); <jats:italic>ASXL1, SETBP1,<\/jats:italic> and <jats:italic>ETNK1<\/jats:italic> mutations did not impact OS. In multivariable analysis, advanced age (<jats:italic>P<\/jats:italic>\u2009=\u2009.003) [age &gt;67: HR 10.1, 95% CI 1.3\u2010119], low hemoglobin (<jats:italic>P<\/jats:italic>\u2009=\u2009.008) [HB&lt; 10 gm\/dL: HR 8.2, 95% CI 1.6\u201023.2] and <jats:italic>TET2<\/jats:italic> mutations (<jats:italic>P<\/jats:italic>\u2009=\u2009.01) [HR 8.8, 95% CI 1.6\u201047.7] retained prognostic significance. We then used age &gt;67 years, hemoglobin &lt;10 gm\/dL and the presence of <jats:italic>TET2<\/jats:italic> mutations (each counted as one risk factor) to create a hazard ratio weighted prognostic model; effectively stratifying patients into two risk categories, low (0\u20101 risk factor) and high (\u22652 risk factors), with median OS of 18 and 7 months, respectively.<\/jats:p>","DOI":"10.1002\/ajh.24722","type":"journal-article","created":{"date-parts":[[2017,3,17]],"date-time":"2017-03-17T16:33:26Z","timestamp":1489768406000},"page":"542-548","source":"Crossref","is-referenced-by-count":77,"title":["Targeted next generation sequencing and identification of risk factors in <scp>W<\/scp>orld <scp>H<\/scp>ealth <scp>O<\/scp>rganization defined atypical chronic myeloid leukemia"],"prefix":"10.1002","volume":"92","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6998-662X","authenticated-orcid":false,"given":"Mrinal M.","family":"Patnaik","sequence":"first","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6768-8987","authenticated-orcid":false,"given":"Daniela","family":"Barraco","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terra L.","family":"Lasho","sequence":"additional","affiliation":[{"name":"Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christy M.","family":"Finke","sequence":"additional","affiliation":[{"name":"Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaaren","family":"Reichard","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine P.","family":"Hoversten","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhett P.","family":"Ketterling","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naseema","family":"Gangat","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayalew","family":"Tefferi","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic Rochester Minnesota USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,4,29]]},"reference":[{"key":"e_1_2_7_2_1","volume-title":"WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues","author":"Swerdlow S","year":"2008"},{"key":"e_1_2_7_3_1","first-page":"1566","article-title":"Identification of risk factors in atypical chronic myeloid leukemia","volume":"91","author":"Breccia 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