ArticleAmerican journal of hematology2021
Clinical and molecular predictors of fibrotic progression in essential thrombocythemia: A multicenter study involving 1607 patients.
Article in American journal of hematology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 39 citations in OpenAlex.
- Review
- Article
- Mutant calreticulin enables potent and selective CAR-T cell therapy in preclinical models of myeloproliferative neoplasms.Journal for immunotherapy of cancer · 2026Article
- Review
- Relationship between additional mutations at diagnosis and treatment response in patients with essential thrombocythemia.Blood advances · 2025Article
- Evolution of WHO diagnostic criteria in "Classical Myeloproliferative Neoplasms" compared with the International Consensus Classification.Blood cancer journal · 2025Review
- HMGA2 overexpression with specific chromosomal abnormalities predominate in CALR and ASXL1 mutated myelofibrosis.Leukemia · 2025Article
- Article
- Article
- One thousand patients with essential thrombocythemia: the Florence-CRIMM experience.Blood cancer journal · 2024Article
- One thousand patients with essential thrombocythemia: the Mayo Clinic experience.Blood cancer journal · 2024Article
- Editorial: Biological and clinical implications of the mutational landscape in myeloproliferative neoplasms.Frontiers in oncology · 2024Article
- Case report: Peri-procedural hydroxyurea helps minimize bleeding in patients with Essential Thrombocythemia associated with acquired von Willebrand syndrome.Frontiers in oncology · 2024Article
- Chronic myeloproliferative neoplasms with concomitantFrontiers in cell and developmental biology · 2024Review
- Deepening Our Understanding of the Factors Affecting Landscape of Myeloproliferative Neoplasms: What Do We Know about Them?Cancers · 2023Review
- Biological drivers of clinical phenotype in myelofibrosis.Leukemia · 2023Review
- Phenotypic correlations of CALR mutation variant allele frequency in patients with myelofibrosis.Blood cancer journal · 2023Article
- [How I diagnose and treat essential thrombocythemia].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2023Article
- International Consensus Classification of myeloid and lymphoid neoplasms: myeloproliferative neoplasms.Virchows Archiv : an international journal of pathology · 2023Review
- Review
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Authors and funding
16 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 < 0.001; HR 6.1; 95% CI 3.2-11.7); Mayo Cohort, 16% vs. 7% at 10 years and 44% vs. 25% at 20 years (p < 0.001; HR 2.5; 95% CI 1.6-4.1); and Rome cohort 7.8% vs. 4.6% at 10 years and 31.2% vs. 7.1% at 20 years (p = 0.007, HR 2.7; 95% CI 1.3-5.8). The present study provides practically useful risk signals for fibrotic transformation in ET and facilitates identification of patients who require close monitoring and appropriate counseling.
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