title: Transfusion-dependency at presentation and its acquisition in the first year of diagnosis are both equally detrimental for survival in primary myelofibrosis--prognostic relevance is independent of IPSS or karyotype.
abstract: The International Prognostic Scoring System (IPSS) and karyotype are useful tools for risk stratification in primary myelofibrosis (PMF). We examined the additional prognostic impact of red blood cell transfusion need among 254 consecutive patients (median age, 59 years). Sixty-two patients ( approximately 24%) required transfusions at diagnosis whereas 22 ( approximately 9%) became transfusion-dependent and 170 remained transfusion-independent during the first year postdiagnosis; after a median follow-up of 55 months, the respective median survivals were 35, 25, and 117 months (P < 0.01). Multivariable analysis confirmed the IPSS- and karyotype-independent prognostic weight of transfusion status. Among IPSS intermediate-1 risk patients, overall median survival of 82 months was modified to 60 or 118 months, based on presence or absence of transfusion need, respectively (P < 0.01). The corresponding figures for intermediate-2/high risk patients were 30 and 64 months (P < 0.01). Documented causes of death did not include iron overload. We conclude that transfusion status in PMF downgrades or upgrades prognosis within specific IPSS categories; transfusion need is a marker of aggressive disease biology in PMF, as it is in myelodysplastic syndromes.
authors.affiliation: Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. tefferi.ayalew@mayo.edu
authors.forename: Ayalew
authors.initials: A
authors.lastname: Tefferi
authors.forename: Sergio
authors.initials: S
authors.lastname: Siragusa
authors.forename: Kebede
authors.initials: K
authors.lastname: Hussein
authors.forename: Susan M
authors.initials: SM
authors.lastname: Schwager
authors.forename: Curtis A
authors.initials: CA
authors.lastname: Hanson
authors.forename: Animesh
authors.initials: A
authors.lastname: Pardanani
authors.forename: Francisco
authors.initials: F
authors.lastname: Cervantes
authors.forename: Francesco
authors.initials: F
authors.lastname: Passamonti
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D017707:Erythrocyte Transfusion; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D055728:Primary Myelofibrosis / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D016019:Survival Analysis