Clinical characteristics, prognostic factors, and outcomes of patients with essential thrombocythemia in Japan: the JSH-MPN-R18 study
Clinical characteristics, prognostic factors, and outcomes of patients with essential thrombocythemia in Japan: the JSH-MPN-R18 study
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DOI：
10.1007/s12185-021-03253-0
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发表时间：
2021-11-02
影响因子：
2.1
通讯作者：
Komatsu, Norio
中科院分区：
文献类型：
--
作者：
Hashimoto, Yoshinori;Ito, Tomoki;Komatsu, Norio
我们对根据世界卫生组织分类诊断标准诊断为原发性血小板增多症的日本患者进行了一项大规模的全国性回顾性研究。我们调查了这些患者的临床特征、生存率、血栓出血事件的发生率以及这些事件的危险因素。本研究共分析了1152例患者。诊断时的中位年龄为65岁，中位血小板计数为832 × 10（9）/L，JAK 2 V617 F、CALR和MPL的阳性突变率分别为62.8%、25.1%和4.1%。与欧洲和美国患者相比，日本患者更可能有心血管危险因素，并且不太可能有全身症状，包括可触及的脾肿大。血小板增多症被确定为出血事件和预后的风险因素，但不是血栓事件的风险因素。欧洲和美国报告的预后因素和风险分类通常适用于日本患者。关于转化，继发性骨髓纤维化以时间依赖性方式进展，但在“真正的”ET患者中进展为急性白血病的程度较低。在日本患者中，皮肤癌不太常见，胃肠道癌作为继发性恶性肿瘤更常见，表明种族差异。
We conducted a large-scale, nationwide retrospective study of Japanese patients who were diagnosed with essential thrombocythemia based on the diagnostic criteria in the World Health Organization classification. We investigated clinical characteristics, survival rates, and the incidence of thrombohemorrhagic events as well as risk factors for these events. A total of 1152 patients were analyzed in the present study. Median age at diagnosis was 65 years, the median platelet count was 832 x 10(9)/L, and the positive mutation rates of JAK2V617F, CALR, and MPL were 62.8, 25.1, and 4.1%, respectively. Compared with European and American patients, Japanese patients were more likely to have cardiovascular risk factors and less likely to have systemic symptoms including palpable splenomegaly. Thrombocytosis was identified as a risk factor for hemorrhagic events and prognosis, but not for thrombotic events. The prognostic factors and risk classifications reported in Europe and the United States were generally applicable to Japanese patients. Regarding transformations, secondary myelofibrosis progressed in a time-dependent manner, but progression to acute leukemia was low in "true" ET patients. Skin cancers were less common and gastrointestinal cancers more common as secondary malignancies in Japanese patients, suggesting ethnic differences.