title: Postpolycythaemic myelofibrosis: frequency and risk factors for this complication in 116 patients.
abstract: Postpolycythaemic myelofibrosis (PPMF) is a known complication of polycythaemia vera (PV) but information regarding its incidence and predisposing factors is not well defined. In 116 subjects consecutively diagnosed with PV in a single institution (median age 62 years, range: 20-88), the probability of PPMF was analysed by the Kaplan-Meier method, followed by the log-rank test. With a mean follow-up of 8 years (95% confidence interval: 6.6-9), 17 patients had evolved into PPMF (15%). The probability of evolution to PPMF was 16% at 10 years and 34% at 15 years. Age, gender, spleen size, leucocytosis, thrombocytosis or cytoreductive treatment were not associated with an increased risk of PPMF. The actuarial probability of PPMF at 15 years was higher in those patients presenting at diagnosis with endogenous megakaryocytic colony formation (59% when present versus 10% when absent, P = 0.03), an elevated serum lactate dehydrogenase (LDH) level (69% vs. 23% in patients with normal LDH, P = 0.04), and in those who were heterozygous for the JAK2 V617F mutation (55% vs. 17% in heterozygotes, P = 0.04). In conclusion, PPMF is a frequent complication in PV patients at 15 years with the risk being higher in patients with increased LDH, endogenous megakaryocytic colony formation or a high JAK2 V617F allele burden.
authors: Haematology Department, Hospital del Mar, IMIM, Barcelona, Spain. 95967@imas.imim.es
authors: Alberto
authors: A
authors: Alvarez-Larrán
authors: Beatriz
authors: B
authors: Bellosillo
authors: Luz
authors: L
authors: Martínez-Avilés
authors: Silvia
authors: S
authors: Saumell
authors: Antonio
authors: A
authors: Salar
authors: Eugenia
authors: E
authors: Abella
authors: Eva
authors: E
authors: Gimeno
authors: Sergi
authors: S
authors: Serrano
authors: Lourdes
authors: L
authors: Florensa
authors: Blanca
authors: B
authors: Sánchez
authors: Carmen
authors: C
authors: Pedro
authors: Carles
authors: C
authors: Besses
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006579:Heterozygote; D006801:Humans; D015994:Incidence; D053614:Janus Kinase 2 / Q000235:genetics; D053208:Kaplan-Meier Estimate; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D008533:Megakaryocytes / Q000473:pathology; D008875:Middle Aged; D009154:Mutation; D011087:Polycythemia Vera / Q000150:complications* / Q000235:genetics / Q000473:pathology; D055728:Primary Myelofibrosis / Q000150:complications* / Q000235:genetics / Q000473:pathology; D011336:Probability; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase; D053614:Janus Kinase 2