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- Title
Comparison of Clinical and Molecular Features Between Patients With Essential Thrombocythemia and Early/Prefibrotic Primary Myelofibrosis Presenting With Thrombocytosis in Taiwan.
- Authors
Kuo, Ming-Chung; Chuang, Wen-Yu; Chang, Hung; Lin, Tung-Huei; Wu, Jin-Hou; Lin, Tung-Liang; Ou, Che-Wei; Hung, Yu-Shin; Huang, Ting-Yu; Huang, Ying-Jung; Wang, Po-Nan; Shih, Lee-Yung
- Abstract
Objectives The clinical presentations of essential thrombocythemia (ET) may be quite similar to early/prefibrotic primary myelofibrosis (pre-PMF), especially in pre-PMF presenting with thrombocytosis (pre–PMF-T), but may be associated with a different outcome. It is very important to distinguish these two entities. The aim of this study was to address the clinical and prognostic relevance of distinguishing pre–PMF-T from ET. Methods All patients, including 258 with ET and 105 with pre–PMF-T, received JAK2 V617F, MPL (exon 10), and CALR (exon 9) mutation analysis and allele burden measurement for JAK2 V617F and CALR mutants. Results Patients with pre–PMF-T had an older age and higher leukocyte and platelet counts but lower hemoglobin levels than patients with ET. Patients with pre–PMF-T had a shorter overall, leukemia-free, and thrombosis-free survival compared with patients with ET. Patients with ET had a higher rate of cerebral ischemic stroke, whereas patients with pre–PMF-T tended to have splanchnic vein thrombosis. The frequencies of JAK2 V617F, CALR , and MPL mutations and CALR allele burden were no different, but JAK2 V617F allele burden was significantly higher in pre–PMF-T. Patients with pre–PMF-T with the JAK2 V617F mutation had an inferior overall survival and thrombosis-free survival, whereas the status of driver gene mutations did not influence the outcomes of patients with ET. Conclusions ET and pre–PMF-T were two distinct disease entities and exhibited different clinical phenotype, genotype, and outcomes.
- Subjects
TAIWAN; MYELOFIBROSIS; THROMBOCYTOSIS; STROKE; THROMBOPOIETIN receptors; LEUKOCYTE count; ISCHEMIC stroke
- Publication
American Journal of Clinical Pathology, 2023, Vol 159, Issue 5, p474
- ISSN
0002-9173
- Publication type
Article
- DOI
10.1093/ajcp/aqac173