title: Significance of cytogenetic abnormalities in patients with polycythemia vera.
abstract: We analyzed 133 patients with polycythemia vera (PV) who were followed at our institution (median 7.5 years) and had adequate cytogenetics information. The 5-, 10- and 15-year survival rates were 93%, 79% and 64%, respectively, with a median projected overall survival of 24 years. Nineteen patients (14%) had abnormal cytogenetics at any time during the disease course (no survival difference). Sixteen patients (12%) underwent disease transformation during follow-up, after a median of 8.5 years, to myelofibrosis (n = 11), acute myeloid leukemia (n = 4) or myelodysplastic syndrome (n = 1); eight had cytogenetic abnormalities. Among 133 patients, 39 were newly diagnosed: 33 with normal and six with abnormal cytogenetics (no survival difference); nine underwent disease transformation (six with normal and three with abnormal cytogenetics at diagnosis). In keeping with other smaller series, the presence of chromosomal abnormalities may have had a role in disease transformation in patients with PV; survival was not affected likely due to short follow-up.
authors.affiliation: Department of Hematology, University Medical Center Ljubljana , Ljubljana , Slovenia.
authors.forename: Matjaz
authors.initials: M
authors.lastname: Sever
authors.forename: Alfonso
authors.initials: A
authors.lastname: Quintás-Cardama
authors.forename: Sherry
authors.initials: S
authors.lastname: Pierce
authors.forename: Lingsha
authors.initials: L
authors.lastname: Zhou
authors.forename: Hagop
authors.initials: H
authors.lastname: Kantarjian
authors.forename: Srdan
authors.initials: S
authors.lastname: Verstovsek
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002471:Cell Transformation, Neoplastic; D002869:Chromosome Aberrations; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D011087:Polycythemia Vera / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D055815:Young Adult