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首页> 外文期刊>American Journal of Case Reports >Triple-Negative Essential Thrombocythemia Complicated by Thrombosis and Acquired von Willebrand Disease in a Young Man
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Triple-Negative Essential Thrombocythemia Complicated by Thrombosis and Acquired von Willebrand Disease in a Young Man

机译：血栓形成和血栓形成的三负基本血小板血症在一个年轻人中获得的冯维尔布朗疾病

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Patient: Male, 25-year-old Final Diagnosis: Acquired von Willebrand deficiency Symptoms: Bleeding ? chest discomfort Medication: — Clinical Procedure: Bleeding ? chest discomfort Specialty: Laboratory Diagnostics ? Hematology ? General and Internal Medicine ? Physiology Objective: Rare co-existance of disease or pathology Background: Essential thrombocythemia (ET) is a type of myeloproliferative neoplasm (MPN) characterized by sustained thrombocytosis in peripheral blood. Patients typically have gene mutations like JAK2 V617F, CALR , and MPL W515L/K. This report describes a young man with ET without any of the above mutations who had paradoxical bleeding due to acquired Von-Willebrand disease. Case Report: A young man with a medical history of thrombocytosis on aspirin presented with acute chest pain and was found to have had a myocardial infarction. Emergency cardiac catheterization revealed a thrombotic occlusion of the left anterior descending (LAD) artery and the right posteriolateral system, with an ejection fraction of 25%. He underwent thrombectomy and balloon angioplasty with LAD stenting, and an Impella 2.5 was inserted due to severe left ventricular dysfunction with akinesia. Aspirin and ticagrelor were administered, but the patient later experienced postoperative bleeding from the site of the Impella device. The bleeding was attributed to acquired Von-Willebrand disease secondary to ET. Emergency plateletpheresis was recommended. Further workup demonstrated that he was triple-negative for JAK2 , MPL , and CALR gene mutations. Conclusions: The paradoxical bleeding resulting from acquired Von-Willebrand disease was likely an entirely separate entity from the hyper-thrombotic state expected from ET. Careful assessment of clinical symptoms and laboratory markers, in addition to a high degree of suspicion, are needed to diagnose acquired Von-Willebrand disease as a complication of ET.
机译：患者：男，25岁的最终诊断：获得的冯维尔布朗缺乏症状：出血？胸部不适药物： - 临床手术：出血？胸部不适专业：实验室诊断？血液学？一般和内科？生理目标：罕见的疾病或病理学背景：基本血小板（ET）是一种肌酚瘤（MPN），其特征在于外周血中持续的血小板效果。患者通常具有JAK2 V617F，CALR和MPL W515L / K等基因突变。本报告描述了一个患有ET等的年轻人，没有任何上述突变，因获得von-willebrand疾病而矛盾的出血。案例报告：一个具有血小板病病史的年轻人对阿司匹林患有急性胸痛的阿司匹林，发现已经有心肌梗死。应急心导管插入件显示左前期下降（LAD）动脉和右后双侧系统的血栓形成闭塞，射血分列为25％。他接受了血栓切除术和气球血管成形术，并且由于患有严重的左心室功能障碍而导致偶像2.5。施用阿司匹林和TiCagreloLor，但患者后来经历了术后从偶像装置的部位出血。出血归因于获得的von-willebrand疾病。建议使用紧急血小板施。进一步的工作证明他是JAK2，MPL和CALR基因突变的三重阴性。结论：由于von-willebrand疾病所引起的矛盾出血可能是预期的超血栓形成的完全单独的实体。除了高度怀疑之外，还要仔细评估临床症状和实验室标志物，以诊断获得的von-willebrand疾病是ET的复杂性。

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