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Philadelphia‐negative myeloproliferative neoplasms among Kuwaiti Nationals

机译:费城 - 阴性肌鳞状肿瘤Kuwaiti国民

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摘要

The epidemiology, genetics, and thrombosis risk of MPNs among Arabs are largely unknown. This may be attributed to scarce epidemiological data, particularly from our region. Our study included 381 Kuwaiti nationals with Ph‐negative MPNs and a confirmed driver mutation involving JAK2 (exon 12 14), CALR, or MPL. This first regional study examines the demographics, clinical parameters, and thrombosis‐related attributes of the participants. This study reported a median age of 58 years, with females and males representing 54.9% and 45.1%, respectively. ET was the most frequent subtype of Ph‐negative MPNs in our population, accounting for 52.0% of the cases, followed by PV, found in 34.6% of the participants, and PMF, found in 8.4% of participants. The crude annual cumulative incidence of Ph‐negative MPNs in Kuwait ranged from 0.674 to 3.177 per 100,000 population across the study period. The most common driver mutation was JAK2V617F, with a frequency of 89.5%. At diagnosis, 19.2% of the patients presented with unexplained thrombosis, and almost half were of arterial origins. Males were more likely to present with arterial thrombosis than females (61.5% vs. 35.3%), whereas venous thrombotic events were more common in females than in males (47.1% vs. 17.9%; p‐value = 0.025). Ph‐negative MPNs in Kuwait are rare; however, thrombosis is a frequent complication, being documented in up to 19.2% of cases at presentation, more commonly at arterial sites. These findings call for thorough evaluation of patients with unexplained derangements in their hematological parameters during follow‐ups.
机译:阿拉伯人中MPN的流行病学,遗传学和血栓形成风险在很大程度上是未知数。这可能归因于稀缺流行病学数据,特别是来自我们的地区。我们的研究包括381个科威特国民,具有pH阴性MPN和涉及JAK2(外显子1214),CALR或MPL的确认驾驶员突变。第一个区域研究审查了参与者的人口统计学,临床参数和血栓形成相关属性。本研究报告称年龄为58岁,女性和雄性分别为54.9%和45.1%。 ET是我们人口中最常见的pH阴性MPN的亚型,占52.0%的案件,其次是PV,在34.6%的参与者和PMF中发现,在8.4%的参与者中发现。科威特PH-Digal MPN的粗累积率为每10万人在研究期间的0.674至3.177。最常见的驾驶员突变是JAK2V617F,频率为89.5%。在诊断中,19.2%的患者呈现出不明原因的血栓形成,几乎一半的动脉起源。男性更有可能呈现动脉血栓形成,而不是女性(61.5%vs.35.3%),而静脉血栓形成在女性中比男性更常见(47.1%与17.9%; p值= 0.025)。科威特的pH阴性MPN是罕见的;然而,血栓形成是一种频繁的并发症,在介绍的患者中达到了高达19.2%的情况,更常见于动脉位点。这些调查结果要求在随访期间对其血液学参数进行彻底评估患有无法解释的紊乱的患者。

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