Revised cytogenetic risk stratification in primary myelofibrosis: analysis based on 1002 informative patients

Leukemia
Ayalew TefferiAnimesh Pardanani

Abstract

Current cytogenetic risk stratification in primary myelofibrosis (PMF) is two-tiered: 'favorable' and 'unfavorable'. Recent studies have suggested prognostic heterogeneity within the unfavorable risk category. In 1002 consecutive patients, we performed stepwise analysis of impact on survival from individual and prognostically ordered cytogenetic abnormalities, leading to a revised three-tiered risk model: 'very high risk (VHR)'-single/multiple abnormalities of -7, i(17q), inv(3)/3q21, 12p-/12p11.2, 11q-/11q23, or other autosomal trisomies not including + 8/ + 9 (e.g., +21, +19); 'favorable'-normal karyotype or sole abnormalities of 13q-, +9, 20q-, chromosome 1 translocation/duplication or sex chromosome abnormality including -Y; 'unfavorable'-all other abnormalities. Median survivals for VHR (n = 75), unfavorable (n = 190) and favorable (n = 737) risk categories were 1.2 (HR 3.8, 95% CI 2.9-4.9), 2.9 (HR 1.7, 95% CI 1.4-2.0) and 4.4 years and survival impact was independent of clinically derived prognostic systems, driver and ASXL1/SRSF2 mutations. The revised model was also effective in predicting leukemic transformation: HRs (95% CI) were 4.4 (2.0-9.4) for VHR and 2.0 (1.2-3.4) for unfavorable. The impact of driver mutations ...Continue Reading

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Nov 30, 2016·Blood Advances·Ayalew TefferiAnimesh Pardanani

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Citations

Jun 5, 2018·Expert Review of Hematology·Elisa RumiUNKNOWN Associazione Italiana per la Ricerca sul Cancro Gruppo Italiano Malattie Mieloproliferative Investigators
Aug 2, 2018·Blood Cancer Journal·Ayalew TefferiAlessandro M Vannucchi
Aug 30, 2018·American Journal of Hematology·Natasha SzuberAyalew Tefferi
Jul 25, 2018·American Journal of Hematology·Ayalew Tefferi
Dec 6, 2018·American Journal of Hematology·Domenico PennaAyalew Tefferi
Sep 20, 2019·Bone Marrow Transplantation·Mario TiribelliMassimiliano Bonifacio
Nov 14, 2019·American Journal of Hematology·Emanuela Sant'AntonioAlessandro Maria Vannucchi
Jan 17, 2020·British Journal of Haematology·Ayalew TefferiAlessandro M Vannucchi
Oct 21, 2019·International Journal of Hematology·Prithviraj Bose, Srdan Verstovsek
Mar 21, 2020·British Journal of Haematology·Naseema Gangat, Ayalew Tefferi
May 21, 2020·Blood·Andrew J DunbarRoss Levine
Apr 15, 2018·Leukemia·Ayalew TefferiAlessandro M Vannucchi
Nov 21, 2018·Blood Cancer Journal·Domenico PennaAyalew Tefferi
Nov 12, 2019·Expert Review of Hematology·Nicole M CruzRichard T Silver
Apr 25, 2019·International Journal of Molecular Sciences·Alessandra IurloUmberto Gianelli
Nov 17, 2020·American Journal of Hematology·Ayalew Tefferi
Dec 2, 2020·International Journal of Molecular Sciences·Elisa RumiEmanuela Sant'Antonio
Dec 10, 2020·OncoTargets and Therapy·Giuseppe G LoscoccoAlessandro M Vannucchi
Mar 2, 2021·Hematology/oncology Clinics of North America·Marta B Davidson, Vikas Gupta
Mar 7, 2021·Pathology·David M RossPiers Blombery
May 31, 2021·Transplantation and Cellular Therapy·Jan Philipp BewersdorfMaximilian Stahl
Jul 22, 2021·International Journal of Laboratory Hematology·Dong ChenOlga Weinberg
Aug 20, 2021·British Journal of Haematology·Nicholas C P CrossUNKNOWN A British Society for Haematology Good Practice Paper
Nov 14, 2021·Current Hematologic Malignancy Reports·Erika Morsia, Naseema Gangat
Jul 22, 2020·Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation·H Joachim DeegBarry E Storer

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