Kamiunten Ayako, Shide Kotaro, Kameda Takuro, Ito Masafumi, Sekine Masaaki, Kubuki Yoko, Hidaka Tomonori, Akizuki Keiichi, Tahira Yuki, Toyama Takanori, Kawano Noriaki, Marutsuka Kousuke, Maeda Kouichi, Takeuchi Masanori, Kawano Hiroshi, Sato Seiichi, Ishizaki Junzo, Shimoda Haruko, Yamashita Kiyoshi, Matsuoka Hitoshi, Shimoda Kazuya
Department of Gastroenterology and Hematology, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki, 889-1692, Japan.
Japanese Red Cross Nagoya Daiichi Hospital, 3-35 Michishita-cho, Nakamura ku, Nagoya, 453-8511, Japan.
Int J Hematol. 2018 Oct;108(4):411-415. doi: 10.1007/s12185-018-2495-2. Epub 2018 Jul 9.
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.
一种新的实体，即早期/纤维化前原发性骨髓纤维化（PMF），在2016年修订的世界卫生组织（WHO）骨髓增殖性肿瘤（MPN）标准中被引入作为PMF的一个亚型。它是根据骨髓（BM）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）值升高和脾肿大]来诊断的。本研究的目的是评估先前诊断为ET的患者中早期/纤维化前PMF的患病率，并比较早期/纤维化前PMF与原发性血小板增多症（ET）患者在诊断时的临床特征和预后。42例患者有ET诊断时获得的BM活检样本。根据2016年修订的WHO标准进行样本重新评估显示，早期/纤维化前PMF占先前诊断为ET的患者的14%，这与先前报告中的发生率相当。与ET患者相比，早期/纤维化前PMF患者的LDH值更高，脾肿大的频率更高。总体而言，两组间无骨髓纤维化生存期和无急性髓系白血病生存期相当。需要准确诊断以明确日本ET患者的临床特征。
