title: Primary chronic myelofibrosis: clinical and prognostic evaluation in 336 Japanese patients.
abstract: We retrospectively analyzed 336 patients with primary chronic myelofibrosis from 203 medical institutes in Japan. Notwithstanding their heterogeneous treatments, the median survival in 298 patients that could be evaluated was 10.0 years. Average age at onset was 60.7 years. Men were affected 1.4 times more frequently than women. The factors associated with shorter survival included anemia, leukocytosis/leukocytopenia, thrombocytopenia, and increased blasts in the peripheral blood, and sex (male), age (>60), and the presence of symptoms. A new scoring system based on the peripheral blood findings (hemoglobin [Hb] level, platelet count, and rate of blast formation) at initial diagnosis clearly correlated with survival rate. Accordingly, patients could be categorized into 3 groups by severity grading. Through stepwise multivariate survival analysis, the significant prognostic factors were identified as the severity grading based on the new scoring system (P = .0002), age (P = .0024), sex (P = .0153), and Hb (P = .0198). Chromosomal abnormalities were found in 58 of 154 patients (38%), although these did not influence survival.
authors.affiliation: First Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan. 1009taok@intmed1.med.kyushu-u.ac.jp
authors.forename: T
authors.initials: T
authors.lastname: Okamura
authors.forename: N
authors.initials: N
authors.lastname: Kinukawa
authors.forename: Y
authors.initials: Y
authors.lastname: Niho
authors.forename: H
authors.initials: H
authors.lastname: Mizoguchi
mesh_terms: D000206:Actuarial Analysis; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000453:epidemiology; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D012189:Retrospective Studies; D012737:Sex Factors; D015996:Survival Rate