title: Chronic myelomonocytic leukemia evolving from preexisting myelodysplasia shares many features with de novo disease.
abstract: The majority of chronic myelomonocytic leukemia (CMML) cases arise de novo; cases evolving from preexisting myelodysplasia (MDS) or myeloproliferative diseases have not been well-studied. We conducted the present study to determine the clinicopathologic features and to study possible underlying molecular and cytogenetic mechanisms involved in this evolution. Between April 1995 and November 2005, we identified 120 CMML cases, of which 20 (16.7%) had a previous diagnosis of MDS. Of the 20 patients with MDS, 6 had relative monocytosis at diagnosis. At the time of MDS to CMML evolution, mutations in JAK2 (V617F), FLT3 (ITD), K-ras-2, or N-ras were not acquired, and only 1 (6%) of 17 evaluable cases showed cytogenetic progression. The median time to evolution from MDS to CMML was 29 months, and the median survival following CMML development was 13 months. Three cases (17%) transformed to acute myeloid leukemia. These findings indicate that in some cases of otherwise typical MDS, the progenitor cells may have some capacity for monocytic proliferation at diagnosis and manifest rapid disease progression once a monocytic proliferation supervenes.
authors.affiliation: Department of Medicine, University of Massachusetts Medical Center, Worcester, MA 01605, USA.
authors.forename: Sa A
authors.initials: SA
authors.lastname: Wang
authors.forename: Naomi
authors.initials: N
authors.lastname: Galili
authors.forename: Jan
authors.initials: J
authors.lastname: Cerny
authors.forename: Eric
authors.initials: E
authors.lastname: Sechman
authors.forename: Su Sin
authors.initials: SS
authors.lastname: Chen
authors.forename: Jerome
authors.initials: J
authors.lastname: Loew
authors.forename: Qin
authors.initials: Q
authors.lastname: Liu
authors.forename: Oluwole
authors.initials: O
authors.lastname: Fadare
authors.forename: Robert
authors.initials: R
authors.lastname: Hasserjian
authors.forename: Dan
authors.initials: D
authors.lastname: Jones
authors.forename: Huma
authors.initials: H
authors.lastname: Qawi
authors.forename: Bruce
authors.initials: B
authors.lastname: Woda
authors.forename: Azra
authors.initials: A
authors.lastname: Raza
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D004247:DNA / Q000737:chemistry / Q000235:genetics; D004252:DNA Mutational Analysis; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D015477:Leukemia, Myelomonocytic, Chronic / Q000209:etiology / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D009196:Myeloproliferative Disorders / Q000150:complications*; D009436:Neural Tube Defects / Q000150:complications*; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics; D018631:ras Proteins / Q000235:genetics
chemical_list: D004247:DNA; C497970:FLT3 protein, human; D051941:fms-Like Tyrosine Kinase 3; C507924:JAK2 protein, human; D053614:Janus Kinase 2; D018631:ras Proteins