The classical Philadelphia chromosome-negative myeloproliferative neoplasms(MPNs),including polycythemia vera,essential thrombocythemia(ET),and primary myelofibrosis,are clonal hematopoietic disorders driven by somati...The classical Philadelphia chromosome-negative myeloproliferative neoplasms(MPNs),including polycythemia vera,essential thrombocythemia(ET),and primary myelofibrosis,are clonal hematopoietic disorders driven by somatic mutations in JAK2(most notably V617F),CALR,or MPL,all of which converge on constitutive activation of the JAK-STAT pathway(1).Triple-negative essential thrombocythemia(TN-ET)is a distinct subtype of ET,accounting for approximately 10%-20%of all ET cases.These cases lack MPN driver mutations but still exhibit histopathological and clinical features sufficient for the diagnosis of ET.A subset of TN-ET cases shows familial predisposition and is caused by germline mutations in JAK2 and MPL(2).Unlike the common somatic V617F mutation,these germline variants are often atypical,located outside classic mutational hotspots,and display variable penetrance(3).展开更多
目的评价口服非肽类促血小板生成素受体激动剂(TPO-RAs)对成年免疫性血小板减少症患者肝酶的影响。方法检索PubMed、Web of Science、中国知网、万方数据和中华医学期刊全文数据库,收集口服非肽类TPO-RAs(干预组)对比安慰剂或常规治疗(...目的评价口服非肽类促血小板生成素受体激动剂(TPO-RAs)对成年免疫性血小板减少症患者肝酶的影响。方法检索PubMed、Web of Science、中国知网、万方数据和中华医学期刊全文数据库,收集口服非肽类TPO-RAs(干预组)对比安慰剂或常规治疗(对照组)的随机对照试验(RCT),检索时限为建库起至2025年6月。筛选文献、提取数据和评价纳入文献质量后,采用RevMan 5.4.1软件进行Meta分析。结果共纳入12项RCT,总计1388例患者,其中干预组971例,对照组417例。Meta分析结果显示,两组患者的肝酶升高发生率[OR=1.24,95%CI(0.77,1.99),P=0.37]、治疗时间≥6周的肝酶升高发生率[OR=1.21,95%CI(0.73,1.99),P=0.46]及重度肝酶升高发生率[OR=1.39,95%CI(0.46,4.20),P=0.55]比较,差异均无统计学意义。亚组分析结果显示,干预组中使用艾曲泊帕[OR=1.57,95%CI(0.85,2.87),P=0.15]、阿伐曲泊帕[OR=0.88,95%CI(0.09,8.46),P=0.91]和海曲泊帕[OR=1.04,95%CI(0.30,3.65),P=0.95]的患者肝酶升高发生率与对照组比较,差异均无统计学意义。结论口服非肽类TPO-RAs不会显著增加成年免疫性血小板减少症患者的肝酶升高发生风险,且整体肝脏安全性良好。展开更多
目的:探讨血液透析患者药物相关血小板减少的发生规律和临床特征,为临床实践提供循证依据。方法:计算机检索中国知网、万方等中文数据库以及PubMed、Embase及Web of Science等外文数据库,收集关于血液透析患者药物相关血小板减少的相关...目的:探讨血液透析患者药物相关血小板减少的发生规律和临床特征,为临床实践提供循证依据。方法:计算机检索中国知网、万方等中文数据库以及PubMed、Embase及Web of Science等外文数据库,收集关于血液透析患者药物相关血小板减少的相关个例报道,并进行描述和分析。结果:共纳入40篇文献(中文26篇、英文14篇),涉及患者41例,患者中位年龄为60岁(16~89岁),男女比例为19∶22(46.34%vs.53.66%),32例(78.05%)主要引发血小板下降的药物包括抗凝药(27例,65.85%)、抗生素(9例,21.95%)、疫苗(2例,4.88%)、喹硫平(1例,2.44%)、烟酸戊四醇酯(1例,2.44%)及头孢西丁及生物不相容性透析器(1例,2.44%);32例(78.05%)的患者血小板减少在15 d及以内发生;39例(95.12%)患者血小板计数下降至100×109 L-1以下,最低者<1×109 L-1。实验室检查显示13例(31.71%)D-二聚体升高,9例(21.95%)肝素诱导的血小板减少症(HIT)抗体阳性,2例(4.88%)抗血小板因子4(PF4)抗体阳性。治疗措施主要以停用可疑药物,改用其他抗凝剂及抗生素,辅以升血小板药物。38例(92.68%)患者治愈,3例(7.32%)患者死亡。结论:血液透析后血小板计数<100×109 L-1,D-二聚体升高,HIT抗体阳性或抗PF4抗体阳性,高度提示血液透析药物相关血小板减少的发生,尤其是抗凝药及抗生素的潜在影响。展开更多
基金supported by Peking University International Hospital Research Grant(No.YN2023QN01).
摘要The classical Philadelphia chromosome-negative myeloproliferative neoplasms(MPNs),including polycythemia vera,essential thrombocythemia(ET),and primary myelofibrosis,are clonal hematopoietic disorders driven by somatic mutations in JAK2(most notably V617F),CALR,or MPL,all of which converge on constitutive activation of the JAK-STAT pathway(1).Triple-negative essential thrombocythemia(TN-ET)is a distinct subtype of ET,accounting for approximately 10%-20%of all ET cases.These cases lack MPN driver mutations but still exhibit histopathological and clinical features sufficient for the diagnosis of ET.A subset of TN-ET cases shows familial predisposition and is caused by germline mutations in JAK2 and MPL(2).Unlike the common somatic V617F mutation,these germline variants are often atypical,located outside classic mutational hotspots,and display variable penetrance(3).
摘要目的评价口服非肽类促血小板生成素受体激动剂(TPO-RAs)对成年免疫性血小板减少症患者肝酶的影响。方法检索PubMed、Web of Science、中国知网、万方数据和中华医学期刊全文数据库,收集口服非肽类TPO-RAs(干预组)对比安慰剂或常规治疗(对照组)的随机对照试验(RCT),检索时限为建库起至2025年6月。筛选文献、提取数据和评价纳入文献质量后,采用RevMan 5.4.1软件进行Meta分析。结果共纳入12项RCT,总计1388例患者,其中干预组971例,对照组417例。Meta分析结果显示,两组患者的肝酶升高发生率[OR=1.24,95%CI(0.77,1.99),P=0.37]、治疗时间≥6周的肝酶升高发生率[OR=1.21,95%CI(0.73,1.99),P=0.46]及重度肝酶升高发生率[OR=1.39,95%CI(0.46,4.20),P=0.55]比较,差异均无统计学意义。亚组分析结果显示,干预组中使用艾曲泊帕[OR=1.57,95%CI(0.85,2.87),P=0.15]、阿伐曲泊帕[OR=0.88,95%CI(0.09,8.46),P=0.91]和海曲泊帕[OR=1.04,95%CI(0.30,3.65),P=0.95]的患者肝酶升高发生率与对照组比较,差异均无统计学意义。结论口服非肽类TPO-RAs不会显著增加成年免疫性血小板减少症患者的肝酶升高发生风险,且整体肝脏安全性良好。