Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia
Early/prefibrotic primary myelofibrosis in patients who were initially diagnosed with essential thrombocythemia
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DOI：
10.1007/s12185-018-2495-2
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发表时间：
2018-10-01
影响因子：
2.1
通讯作者：
Shimoda, Kazuya
中科院分区：
文献类型：
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作者：
Kamiunten, Ayako;Shide, Kotaro;Shimoda, Kazuya
在2016年修订的世界卫生组织(WHO)骨髓增生性肿瘤(MPN)标准中，引入了一个新的实体，即早期/纤维化前原发性骨髓纤维化(PMF)，作为PMF的一个亚型。诊断依据是骨髓活检标本的组织病理学特征以及临床参数[白细胞增多、贫血、乳酸脱氢酶(LDH)升高和脾肿大]。本研究的目的是评估早期/纤维化前期PMF在既往诊断为ET的患者中的患病率，并比较早期/纤维化PMF与原发性血小板增多症(ET)患者的临床特征和预后。42例患者在ET诊断时获得骨髓活检标本。根据2016年修订的WHO标准进行的样本重新评估显示，早期/纤维化前期PMF占以前诊断为ET的患者的14%，与以前报告中的比率相当。与ET患者相比，早期/纤维化前期PMF患者的LDH值更高，脾肿大的发生率更高。总体而言，两组之间无骨髓纤维化和无急性髓系白血病的存活率是相似的。需要准确的诊断以明确日本ET患者的临床特征。
A new entity, namely early/prefibrotic primary myelofibrosis (PMF), was introduced as a subtype of PMF in the 2016 revised World Health Organization (WHO) criteria for myeloproliferative neoplasms (MPN). It was diagnosed based on histopathological features of bone marrow (BM) biopsy specimens together with clinical parameters [leukocytosis, anemia, elevated lactate dehydrogenase (LDH) values, and splenomegaly]. The aim of this study was to evaluate the prevalence of early/prefibrotic PMF in patients who were previously diagnosed with ET, and to compare clinical features at diagnosis and outcomes between early/prefibrotic PMF and essential thrombocythemia (ET) patients. BM biopsy samples obtained at the time of ET diagnosis were available in 42 patients. Sample reevaluation according to the 2016 revised WHO criteria revealed that early/prefibrotic PMF accounted for 14% of patients who were previously diagnosed with ET, which was comparable to the rates in previous reports. Compared to patients with ET, patients with early/prefibrotic PMF had higher LDH values and higher frequencies of splenomegaly. Overall, myelofibrosis-free and acute myeloid leukemia-free survivals were comparable between the 2 groups. Accurate diagnosis is required to clarify the clinical features of Japanese ET patients.