{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,17]],"date-time":"2026-06-17T13:04:14Z","timestamp":1781701454507,"version":"3.54.5"},"reference-count":81,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2002,10,3]],"date-time":"2002-10-03T00:00:00Z","timestamp":1033603200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2002,10,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>BACKGROUND<\/jats:title><jats:p>Up to 5% of patients with chronic myelogenous leukemia (CML) do not have the Philadelphia (Ph) translocation t(9;22)(q34;q11) or a <jats:italic>bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> molecular rearrangement. Although the diagnostic criteria of this entity are still under debate, there is general agreement that patients with Ph negative, <jats:italic>bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> negative CML have a severe clinical course that is not affected significantly by current treatment options.<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>A population of 76 patients with <jats:italic>bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> negative CML who had received minimal or no previous therapy was characterized carefully with the intent of investigating clinical and hematologic variables and their association with survival by univariate, correlation, and multivariate analyses. A group of 73 patients with Ph negative CML who were not tested for the <jats:italic>bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> rearrangement (bcr\/abl <jats:italic>unknown<\/jats:italic>) was analyzed separately and used for extension of the analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>In the <jats:italic>bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> negative patient population, the median overall survival was 24 months. At the time of the analysis, 38 patients (50%) had died, and blastic transformation preceded death in 31%. Chromosomal abnormalities were found in 30% of the 76 patients, with trisomy 8 the most common abnormality. Complex chromosomal abnormalities were rare, and monosomy 7 was not observed. Survival was not affected significantly by treatment. Multivariate analysis identified older age (&gt; 65 years), anemia (hemoglobin &lt; 10 g\/dL), and severe leukocytosis (white blood cells &gt; 50 \u00d7 10<jats:sup>9<\/jats:sup>\/L) as variables with independent prognostic significance for poor survival. A prognostic scoring system stratified patients into a low\u2010risk group (53%) and a high\u2010risk group (47%), with median survivals of 38 months and 9 months, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>CONCLUSIONS<\/jats:title><jats:p><jats:italic>Bcr<\/jats:italic>\/<jats:italic>abl<\/jats:italic> negative CML is a distinct clinical entity associated with very poor prognosis. Two risk categories are identifiable using a simple scoring system based on age, hemoglobin level, and leukocyte number. Cancer 2002;95:1673\u201384. \u00a9 2002 American Cancer Society.<\/jats:p><jats:p>DOI 10.1002\/cncr.10832<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.10832","type":"journal-article","created":{"date-parts":[[2002,10,9]],"date-time":"2002-10-09T01:58:50Z","timestamp":1034128730000},"page":"1673-1684","source":"Crossref","is-referenced-by-count":87,"title":["Characteristics and outcome of patients with Philadelphia chromosome negative, <i>bcr<\/i>\/<i>abl<\/i> negative chronic myelogenous leukemia"],"prefix":"10.1002","volume":"95","author":[{"given":"Francesco","family":"Onida","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greg","family":"Ball","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hagop M.","family":"Kantarjian","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry 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