比较台湾血小板血症基本血症和早期/预纤维化原发性骨髓纤维化患者之间的临床和分子特征的比较
Comparison of Clinical and Molecular Features Between Patients With Essential Thrombocythemia and Early/Prefibrotic Primary Myelofibrosis Presenting With Thrombocytosis in Taiwan
影响因子:1.90000
分区:医学4区 / 病理学3区
发表日期:2023 May 02
作者:
Ming-Chung Kuo, Wen-Yu Chuang, Hung Chang, Tung-Huei Lin, Jin-Hou Wu, Tung-Liang Lin, Che-Wei Ou, Yu-Shin Hung, Ting-Yu Huang, Ying-Jung Huang, Po-Nan Wang, Lee-Yung Shih
摘要
必需血小板血症（ET）的临床表现可能与早期/预纤维化原发性骨髓纤维化（PRE-PMF）非常相似，尤其是在PROD-PMF中呈现血小板症（PRED-PMF-T），但可能与不同的结果有关。区分这两个实体非常重要。这项研究的目的是解决将PRMF-T与ET的临床和预后相关性解决的临床和预后相关性，其中包括ET的258例ET和PRE-PMF-T的105，接受JAK2V617F，MPL（Exon 10）和Calr（外显子9）和较高的preem and pre ant pre Mutants and pre Mutants ant pre Mutants ant pre mutant.pmft-pmpf-pmft-pmft-pmft-ptt-p.pt-ptt-pateations and Calr（Exon 9）突变。白细胞和血小板计数，但血红蛋白水平低于ET患者。与ET患者相比，PRE-PMF-T患者的总体总体，无白血病和无血栓形成生存率较短。 ET患者的脑缺血性中风率较高，而PMF-T前患者患有斑点头静脉血栓形成。 JAK2V617F，CALR和MPL突变以及Calr等位基因负担的频率没有什么不同，但是PRE-PMF-T的JAK2V617F等位基因负担明显更高。患有JAK2V617F突变的PRE-PMF-T患者的总生存率较低，无血栓形成无生存期，而驱动基因突变的状态并不影响ET.ET和PMF-T前患者的结果，PMF-T是两个不同的疾病实体，并且表现出不同的临床表型，基因型，基因型和成果。
Abstract
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.All patients, including 258 with ET and 105 with pre-PMF-T, received JAK2V617F, MPL (exon 10), and CALR (exon 9) mutation analysis and allele burden measurement for JAK2V617F and CALR mutants.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 JAK2V617F, CALR, and MPL mutations and CALR allele burden were no different, but JAK2V617F allele burden was significantly higher in pre-PMF-T. Patients with pre-PMF-T with the JAK2V617F 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.ET and pre-PMF-T were two distinct disease entities and exhibited different clinical phenotype, genotype, and outcomes.