Clinical and molecular predictors of fibrotic progression in essential thrombocythemia: A multicenter study involving 1607 patients
In: American Journal of Hematology, Jg. 96 (2021-09-06), S. 1472-1480
Online
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Zugriff:
The current retrospective study involving a total of 1607 patients was designed to identify clinical and molecular variables that were predictive of inferior myelofibrosis-free survival (MFS) in WHO-defined essential thrombocythemia (ET), utilizing three independent patient cohorts: University of Florence, Italy (n = 718); Mayo Clinic, USA (n = 479) and Policlinico Gemelli, Catholic University, Rome, Italy (n = 410). The Florence patient cohort was first examined to identify independent risk factors for MFS, which included age > 60 years (HR 2.5, 95% CI 1.3-4.9), male sex (2.1, 1.2-3.9), palpable splenomegaly (2.1, 1.2-3.9), CALR 1/1-like or MPL mutation (3.4, 1.9-6.1) and JAK2V617F variant allele frequency > 35% (4.2, 1.6-10.8). Subsequently, an operational molecular risk category was developed and validated in the other two cohorts from Mayo Clinic and Rome: "high molecular risk" category included patients with JAK2V617F VAF >35%, CALR type 1/1-like or MPL mutations; all other driver mutation profiles were assigned to "low molecular risk" category. The former, compared to the latter molecular risk category, displayed significantly higher risk of fibrotic transformation: Florence cohort with respective fibrotic transformation risk rates of 8% vs. 1.2% at 10 years and 33% vs. 8% at 20 years (p
Titel: |
Clinical and molecular predictors of fibrotic progression in essential thrombocythemia: A multicenter study involving 1607 patients
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Autor/in / Beteiligte Person: | Tefferi, Ayalew ; Rossi, Elena ; Gangat, Naseema ; Betti, Silvia ; Vannucchi, Alessandro M. ; Mannarelli, Carmela ; Paoli, Chiara ; Ramundo, Francesco ; Maccari, Chiara ; Giuseppe Gaetano Loscocco ; Pardanani, Animesh ; Jadoon, Yamna ; Ceglie, Sara ; Guglielmelli, Paola ; Gesullo, Francesca ; Valerio De Stefano |
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Zeitschrift: | American Journal of Hematology, Jg. 96 (2021-09-06), S. 1472-1480 |
Veröffentlichung: | Wiley, 2021 |
Medientyp: | unknown |
ISSN: | 1096-8652 (print) ; 0361-8609 (print) |
DOI: | 10.1002/ajh.26332 |
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