title: Application of five prognostic survival scores to primary myelofibrosis in 62 Brazilian patients.
abstract: Primary myelofibrosis (PM) is a Philadelphia-negative clonal hematopoietic stem cell disorder characterized by intense reactive changes of bone marrow stroma with collagen fibrosis, osteosclerosis and angiogenesis. PM usually affects elderly people, and approximately half of the patients present JAK2V617F mutation. PM clinical course varies from 1 to 30 years, evolving from asymptomatic into progressive bone marrow failure, symptomatic splenomegaly or acute leukemia in 10-20 % of cases. PM risk stratification is based on parameters predicting survival, and several attempts have been made to identify clinical and laboratory features that could predict PM patient survival. This study applied five prognostic scores: Dupriez, Cervantes, Mayo, IPSS and DIPSS-Plus in 62 Brazilians patients from three centers, and compared their relevance and clinical usefulness considering the scores' parameters, fibrosis, JAK2V617F mutation, splenomegaly, hepatomegaly and treatment. According to the Cervantes, Dupriez and Mayo scores, most patients were stratified into low-risk group. However, when IPSS and DIPSS-Plus were applied, most patients were classified into an intermediate range, being low risk in only 11 and 13 % of patients, respectively. Overall survival at 4 years was 84 %. The Cervantes score was the only one that remained significantly associated with survival in a multivariate analysis. In conclusion, the Cervantes score remains important to the prognostication of PM.
authors.affiliation: Disciplina de Hematologia, Universidade Federal de São Paulo, SP, Brazil.
authors.forename: M C
authors.initials: MC
authors.lastname: Souza
authors.forename: C A
authors.initials: CA
authors.lastname: Rodrigues
authors.forename: M R R
authors.initials: MR
authors.lastname: Silva
authors.forename: J
authors.initials: J
authors.lastname: Ribeiro
authors.forename: R
authors.initials: R
authors.lastname: Tognon
authors.forename: F A
authors.initials: FA
authors.lastname: Castro
authors.forename: B P
authors.initials: BP
authors.lastname: Simões
authors.forename: E X
authors.initials: EX
authors.lastname: Souto
authors.forename: M L
authors.initials: ML
authors.lastname: Chauffaille
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001938:Brazil; D005260:Female; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D055728:Primary Myelofibrosis / Q000145:classification / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics; D011379:Prognosis; D016019:Survival Analysis
chemical_list: C507924:JAK2 protein, human; D053614:Janus Kinase 2