Rana Muhammad Asim, Mady Ahmed F, Lashari Abdullah Ali, Eltreafi Rehab, Fischer-Orr Nicola, Naser Kamal
Critical Care Department, Bahria Town International Hospital, Lahore, Pakistan.
Critical Care Department, King Saud Medical City, Riyadh, Saudi Arabia.
Case Rep Crit Care. 2018 May 27;2018:7273420. doi: 10.1155/2018/7273420. eCollection 2018.
Thrombotic storm (TS) is a rare, acute, hypercoagulable state characterized by multiple thromboembolic events affecting at least two different areas of the vascular system/organs over a short period of time. Typical triggers include inflammation, infections, minor trauma, surgery, pregnancy, and the puerperium. A single thrombotic event can set off a number of thromboembolic events, often including unusual locations like hepatic, portal, or renal veins, skin , adrenal glands, and cerebral sinus venous thrombosis. Usually, younger female patients are affected; in some patients, there is an association with an autoimmune disorder like lupus erythematosus, and they show evidence of antiphospholipid antibodies or other phenotypic expressions of anticoagulation disorders. The majority of patients have no previous history of thromboembolism. As the diagnosis of thrombotic storm relies solely on clinical symptoms with a lack of specific diagnostic tests, this can result in a delay of diagnosis. The treatment consists of uninterrupted lifelong anticoagulation. Sometimes immunomodulatory therapies have been used. The distinction between extensive thrombotic events like Heparin Induced Thrombosis (HIT), Thrombotic Thrombocytopenic Purpura (TTP), Antiphospholipids Syndrome (APS), and TS can sometimes be difficult, and the etiology of TS remains uncertain.
血栓风暴(TS)是一种罕见的急性高凝状态,其特征是在短时间内发生多个血栓栓塞事件,影响血管系统/器官的至少两个不同区域。典型的触发因素包括炎症、感染、轻微创伤、手术、妊娠和产褥期。单个血栓形成事件可引发一系列血栓栓塞事件,常累及不常见的部位,如肝静脉、门静脉或肾静脉、皮肤、肾上腺以及脑静脉窦血栓形成。通常,年轻女性患者易受影响;部分患者与自身免疫性疾病如红斑狼疮有关,且存在抗磷脂抗体或抗凝障碍的其他表型表现。大多数患者既往无血栓栓塞病史。由于血栓风暴的诊断仅依赖临床症状,缺乏特异性诊断检查,这可能导致诊断延迟。治疗包括终身不间断抗凝。有时也会使用免疫调节疗法。肝素诱导的血栓形成(HIT)、血栓性血小板减少性紫癜(TTP)、抗磷脂综合征(APS)等广泛血栓形成事件与血栓风暴之间的区分有时较为困难,血栓风暴的病因仍不明确。