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Vol. 43. Núm. S1.
Páginas S128 (Outubro 2021)
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Vol. 43. Núm. S1.
Páginas S128 (Outubro 2021)
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CLINICAL, CYTOGENETIC AND MOLECULAR CHARACTERIZATION OF BRAZILIAN COHORT PATIENTS WITH PRIMERY MYELOFIBROSIS AND MYELOFIBROSIS POST-POLYCYTTHEMIA VERA AND POST-ESSENTIAL THROMBOCYTHEMIA
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RS Tavaresa, NCD Clementinob, MRM Conchonc, KBB Pagnanod, SA Santanab, ACKVD Nascimentoe, VAM Funkef, RT Centroneg, FS Seguroh, JAP Bortolinii, DA Silveirah, I Bendith
a Universidade Federal de Goiás (UFG), Goiânia, GO, Brazil
b Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil
c Hospital Santa Marcelina (HSM), São Paulo, SP, Brazil
d Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil
e Irmandade da Santa Casa de Misericórdia de São Paulo, São Paulo, SP, Brazil
f Universidade Federal do Paraná (UFPR), Curitiba, PR, Brazil
g Instituto Hemomed de Oncologia e Hematologia, São Paulo, SP, Brazil
h Universidade de São Paulo (USP), São Paulo, SP, Brazil
i Centro de Pesquisas Oncológicas (CEPON), Florianópolis, SC, Brazil
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Introduction

Myelofibrosis (MF) is the most severe myeloproliferative neoplasia (MPN), with a very variable survival, which can be beyond 20 years to less than two years. The only curative treatment is allogeneic bone marrow transplantation (ABMT), but this is restricted to patients of less advanced age and is associated with significant mortality and disease relapse. Adequate prognostic characterization is essential for therapeutic decision-making, and modern prognostic indexes using molecular and cytogenetic data have been replacing the traditional indexes.

Materials and methods

Clinical and laboratory data were evaluated, bone marrow and peripheral blood karyotype were studied, and BCR-ABL1, JAK2, CALR, MPL, ASXL1, SRSF2, EZH2, IDH1, IDH2, U2AF1 mutations were investigated. The classic IPSS, DIPSS, DIPSS plus, and the most recent MIPSS70, MIPSS70 plus, MIPSS 70 plus 2.0, GIPSS, and MYSEC-PM indexes were applied to a population of 230 patients from 8 Brazilian institutions with primary myelofibrosis (MFP) and secondary myelofibrosis polycythemia vera (PV-MF) and essential thrombocythemia (TE-MF) and compared among themselves.

Results and conclusions

The median-age was 65 years-old, the overall survival was 8.1 years (CI 95%, 7,2 – 10,7), with a 5 years estimated survival of 61% (53 – 70). Comparing the prognostic indexes with each other identified a greater accuracy of the prognostic indexes that use mutational and cytogenetic data on the classic prognostic indexes. In the univariate analysis, advanced age, male gender, spleen size, hemoglobin, white blood cell count, peripheral blood blast percentage, SRSF2 mutation, triple-negative patients, IPSS, DIPSS and DIPSS plus stratification, MIPSS70 high risk and MIPSS70 plus high and very high risk showed impact in survival. In the multivariate analysis, triple-negative for JAK2 V617F, CARL, and MPL (p = 0,002) and stratification by IPSS (p < 0,0001) maintained significance as risk factors for overall survival. The present study is the most extensive Brazilian cohort study of patients with MF evaluating these variables.

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Hematology, Transfusion and Cell Therapy
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