antiphospholipid antibody syndrome

抗磷脂抗体综合征
  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    严重急性呼吸综合征冠状病毒2疫苗接种后的血栓形成是具有血栓性倾向的患者的严重并发症。在这里,我们介绍了一名17岁女性,她患有潜在的抗磷脂综合征(APS),并在第二次接种BNT162b2疫苗6个月后出现了深静脉血栓形成(DVT).虽然她没有家族血栓史,她以前在6岁时患上DVT,右髂总静脉和下腔静脉有血栓形成,伴随左肺梗死。患者在DVT发病后接受抗凝治疗6年,随后停止治疗5年无复发。她在17岁时接种了BNT162b2疫苗,1周前例行门诊就诊。首次接种后14天检测到血小板因子4升高,持续5个月无血栓症状。第二次接种疫苗六个月后,DVT复发,采用直接口服抗凝剂治疗.假设疫苗通过激活凝血来加剧患者的APS。血小板因子4水平可能指示凝血状态。当易患血栓的患者接种疫苗时,应监测凝血状态和血小板活化标志物以预防DVT的发生.
    Thrombosis after severe acute respiratory syndrome coronavirus 2 vaccination is a serious complication in patients with a thrombophilic predisposition. Herein, we present a 17-year-old female who had underlying antiphospholipid syndrome (APS) and developed deep vein thrombosis (DVT) 6 months after her second BNT162b2 vaccine dose. Although she had no family history of thrombosis, she had previously developed DVT at 6 years of age, with thrombus formation in the right common iliac vein and the inferior vena cava, along with concomitant left pulmonary infarction. The patient had received anticoagulant therapy for 6 years after DVT onset, with subsequent treatment cessation for 5 years without recurrence. She received the BNT162b2 vaccine at 17 years of age, 1 week before a routine outpatient visit. Platelet factor 4 elevation was detected 14 days after the first vaccination, persisting for 5 months without thrombotic symptoms. Six months after the second vaccine dose, the DVT recurred and was treated with a direct oral anticoagulant. The vaccine was hypothesized to exacerbate the patient\'s APS by activating coagulation. Platelet factor 4 levels may indicate coagulation status. When patients predisposed to thrombosis are vaccinated, coagulation status and platelet activation markers should be monitored to prevent DVT development.
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  • 文章类型: Case Reports
    一名33岁的女性系统性红斑狼疮患者在5年内表现为二尖瓣疾病的快速进展,强调对轻度至中度瓣膜疾病常规监测指南的关注。
    A 33-year-old woman with systemic lupus erythematosus presented with rapid progression of mitral valve disease within a 5-year period, highlighting concerns regarding routine surveillance guidelines for mild to moderate valvular disease.
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  • 文章类型: Journal Article
    背景:我们旨在调查ACTD孕妇的围产期结局与胎盘病理特征之间的关系,包括系统性红斑狼疮(SLE),抗磷脂抗体综合征(APS),和未分化结缔组织病(UCTD)。
    方法:SLE胎盘组织(n=44),APS(n=45),和UCTD(n=45)包括在内,在2015年9月至2021年3月期间,将同期分娩胎盘作为对照组(n=46).使用人类胎盘病理学手册评估胎盘组织病理学,并根据阿姆斯特丹共识框架进行分类。
    结果:SLE孕妇剖宫产率较高(61.40%),早产(24.56%),与对照组相比,SGA(26.32%)(分别为p=0.008,p=0.005和p=0.000)。血管灌注不良的发生率,炎症-免疫损伤,SLE组其他胎盘病变占47.73%,56.82%,和63.64%,均高于对照组(分别为p=0.000、p=0.000和p=0.006)。同时,APS组炎症-免疫病变的发生率(42.22%,p=0.004)和UCTD组的血管灌注不良(37.78%,与对照组相比,p=0.007)增加。
    结论:SLE似乎增加了围产期各种不良结局的风险。我们确定了大多数ACTD女性胎盘组织病理学风险升高,包括血管发育不良,血管灌注不良,和炎症免疫损伤。
    BACKGROUND: We aimed to investigate the association between perinatal outcomes and placental pathological features in pregnant women with ACTD, including systemic lupus erythematosus (SLE), antiphospholipid antibody syndrome (APS), and undifferentiated connective tissue disease (UCTD).
    METHODS: Placental tissue from SLE (n = 44), APS (n = 45), and UCTD (n = 45) were included, and contemporaneous deliveries of placenta were served as a control group (n = 46) between September 2015 and March 2021. The placental histopathology was evaluated using the Manual of Human Placental Pathology and classified according to the Amsterdam consensus framework.
    RESULTS: SLE pregnant women have a higher rate of cesarean section (61.40%), premature birth (24.56%), and SGA (26.32%) when compared to control group (p = 0.008, p = 0.005, and p = 0.000, respectively). The rate of vascular malperfusion, inflammatory-immune lesions, and other placental lesions in the SLE group was 47.73%, 56.82%, and 63.64%, which were higher than the control group (p = 0.000, p = 0.000, and p = 0.006, respectively). In the meantime, the incidence of inflammatory-immune lesions in the APS group (42.22%, p = 0.004) and vascular malperfusion in the UCTD group (37.78%, p = 0.007) were increased when compared to the control group.
    CONCLUSIONS: SLE appeared to confer increased risk for a wide range of adverse perinatal outcomes. We determined elevated placental histopathology risk for most women with ACTD, including vascular maldevelopment, vascular malperfusion, and inflammatory-immune lesions.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    脑静脉血栓形成(CVT)是一种以脑静脉窦血栓形成为特征的脑血管疾病,导致静脉梗塞。这种情况可以通过一系列的体征和症状表现出来,如头痛,良性颅内高压,蛛网膜下腔出血,局部神经功能缺损,癫痫发作,无法解释的意识变化,和脑膜脑炎.其原因与许多不同的条件和因素有关。我们报告了一名年轻患者的复杂病例和抗磷脂抗体综合征的病程。此案始于两年前,涉及一名因IgA肾病而患有慢性肾病的33岁男子,肺炎,在他的原始二尖瓣上还有一大块.他上肢出现了深静脉血栓(DVT),为此他开了华法林。他被转移到我们医院,有五天的严重头痛史,随后意识下降和癫痫发作需要插管。他被发现患有硬膜下血肿,国际标准化比率(INR)很高。他接受了血肿清除术和右开颅减压术。通过CT静脉造影对大脑进行CT检查显示,脑出血以及右额顶叶和颞叶的缺血性梗塞以及脑静脉血栓形成。他接受了肝素输注治疗,但后来出现了肝素诱导的血小板减少症(HIT),并改用磺达肝素。给予血浆置换和静脉注射甲基强的松龙。他的住院过程因反复感染而变得复杂,新的左脑实质内出血伴脑室扩张,以及需要额外的脑室引流(EVD)。诊断为抗磷脂抗体综合征。该病例报告提供了宝贵的见解,以管理复杂的情况,该情况需要在严重ICH的情况下进行抗凝治疗与免疫抑制治疗的必要性之间进行平衡决策。重点是使用个性化和多学科战略来解决CVT情况及其问题的重要性。
    Cerebral venous thrombosis (CVT) is a cerebrovascular condition characterized by cerebral venous sinus thrombosis, resulting in venous infarction. The condition can manifest through a range of signs and symptoms such as headaches, benign intracranial hypertension, subarachnoid hemorrhage, localized neurological deficits, seizures, unexplained changes in consciousness, and meningoencephalitis. Its causes are linked to numerous different conditions and factors. We report a complicated case and course of antiphospholipid antibody syndrome in a young patient. The case began two years prior, involving a 33-year-old man who had chronic kidney disease due to IgA nephropathy, pneumonia, and a large mass on his native mitral valve. He developed deep vein thrombosis (DVT) in his upper limb, for which he was prescribed warfarin. He was transferred to our hospital with a five-day history of severe headaches followed by a decrease in consciousness and seizures requiring intubation. He was found to have a subdural hematoma with a high international normalized ratio (INR). He underwent hematoma evacuation and a right decompressive craniotomy. CT of the brain via CT venography revealed intracerebral haemorrhage along with ischemic infarction in the right frontal-parietal and temporal lobes and cerebral venous thrombosis. He was treated with heparin infusion but later developed heparin-induced thrombocytopenia (HIT) and was switched to fondaparinux. Plasma exchange and intravenous methylprednisolone were given. His hospital course was complicated by recurrent infections, a new left intraparenchymal hemorrhage with intraventricular extension, and the need for extra ventricular drainage (EVD). The diagnosis of antiphospholipid antibody syndrome was confirmed. This case report provides invaluable insights into managing a complex scenario that requires balanced decisions between anticoagulation in the context of severe ICH and the necessity of immunosuppressive therapy. The emphasis is on the significance of using a personalized and multidisciplinary strategy to address CVT situations and their issues.
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  • 文章类型: Case Reports
    无与抗磷脂抗体综合征相关的粒细胞集落刺激因子产生肺癌的报道。一名73岁的男子被转介到我们部门接受右上叶肺癌手术。他的检查结果表明,他的病情是由白细胞计数升高和粒细胞集落刺激因子产生引起的炎症反应增加引起的。怀疑存在抗磷脂抗体综合征,凝血因子的减少被认为是由狼疮抗凝药抑制的。围手术期,患者接受了肝素和类固醇治疗,并进行了胸腔镜辅助的右上叶切除术。术后,组织病理学检查显示多形性癌,患者抗心磷脂IgG抗体检测呈阴性。在白细胞计数升高的肺癌患者中,发烧,和炎症反应,产生粒细胞集落刺激因子的肺癌是一种重要的鉴别诊断.此外,当术前观察到凝血异常时,彻底的检查是必要的准备围手术期管理。
    No reports on granulocyte colony-stimulating factor-producing lung cancer associated with antiphospholipid antibody syndrome. A 73-year-old man was referred to our department to undergo surgery for lung cancer in the right upper lobe. His examination results suggested that his condition was caused by an elevated white blood cell count and an increased inflammatory response due to granulocyte colony-stimulating factor production. The presence of antiphospholipid antibody syndrome was suspected, and the decrease in coagulation factors was considered to be inhibited by the lupus anticoagulant. Perioperatively, the patient was treated with heparin and steroids, and a thoracoscopically assisted right upper lobectomy was performed. Postoperatively, histopathological examination revealed pleomorphic carcinoma, and the patient tested negative for anticardiolipin IgG antibodies. In lung cancer patients with elevated white blood cell counts, fever, and an inflammatory response, granulocyte colony-stimulating factor-producing lung cancer is an important differential diagnosis. Additionally, when coagulation abnormalities are observed preoperatively, a thorough examination is necessary to prepare for perioperative management.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    血小板在止血中起重要作用,低血小板计数通常会增加出血的风险。尽管血小板计数低但仍发生血栓形成的情况称为血栓形成伴血小板减少综合征。包括肝素诱导的血小板减少症,疫苗诱导的免疫性血栓性血小板减少症,阵发性夜间血红蛋白尿,抗磷脂综合征,血栓性微血管病(TMA),和弥散性血管内凝血。TMA包括血栓性血小板减少性紫癜,产志贺毒素大肠杆菌相关溶血性尿毒综合征(HUS),和非典型的HUS。具有这些病理的患者存在与血小板和凝血系统的活化相关的血栓形成和消耗性血小板减少症。治疗因疾病而异,如果不及时实施治疗干预措施,许多疾病对死亡率和器官预后有直接影响.基础疾病以及体格检查和一般实验室检查的结果,作为对患者进行彻底检查的一部分,应在明确诊断之前迅速进行治疗干预。对于一些疾病,诊断和初始治疗必须并行进行。不仅使用实验室测试,而且使用各种评分系统对于基于临床信息验证治疗干预措施非常重要。
    Platelets play an important role in hemostasis, and a low platelet count usually increases the risk of bleeding. Conditions in which thrombosis occurs despite low platelet counts are referred to as thrombosis with thrombocytopenia syndrome, including heparin-induced thrombocytopenia, vaccine-induced immune thrombotic thrombocytopenia, paroxysmal nocturnal hemoglobinuria, antiphospholipid syndrome, thrombotic microangiopathy (TMA), and disseminated intravascular coagulation. TMA includes thrombotic thrombocytopenic purpura, Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome (HUS), and atypical HUS. Patients with these pathologies present with thrombosis and consumptive thrombocytopenia associated with the activation of platelets and the coagulation system. Treatment varies from disease to disease, and many diseases have direct impacts on mortality and organ prognosis if therapeutic interventions are not promptly implemented. Underlying diseases and the results of physical examinations and general laboratory tests as part of a thorough workup for patients should promptly lead to therapeutic intervention before definitive diagnosis. For some diseases, the diagnosis and initial treatment must proceed in parallel. Utilization of not only laboratory tests but also various scoring systems is important for validating therapeutic interventions based on clinical information.
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  • 文章类型: Case Reports
    一名37岁的女性患有充血性心力衰竭时出现的二尖瓣感染性心内膜炎。然而,当她还被发现患有抗磷脂抗体综合征时,临床情况变得复杂。多学科团队的精心优化和及时的手术干预有助于缓解这种不常见的情况,并导致成功的结果。
    A 37-year-old lady with infective endocarditis of the mitral valve presented in congestive cardiac failure. However, the clinical scenario became complicated when she was also found to have antiphospholipid antibody syndrome. Meticulous optimization and timely surgical intervention by a multidisciplinary team helped mitigate this not so common situation and lead to successful outcome.
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