Mafalda Vasconcelos, Patrícia Moreira, Teresa Costa E Silva, João Espírito Santo, Carla Noronha
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Chronic anticoagulation with enoxaparin, with dosage guided by anti-factor Xa activity, resulted in stability without complications over 17 years. This case emphasizes the importance of personalized therapeutic strategies and close monitoring in patients with APS.</p><p><strong>Learning points: </strong>This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison's disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications.After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose).The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"11 11","pages":"004877"},"PeriodicalIF":0.0000,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11542954/pdf/","citationCount":"0","resultStr":"{\"title\":\"Complex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.\",\"authors\":\"Mafalda Vasconcelos, Patrícia Moreira, Teresa Costa E Silva, João Espírito Santo, Carla Noronha\",\"doi\":\"10.12890/2024_004877\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies and venous and arterial thrombotic events, including obstetric complications. 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引用次数: 0
摘要
抗磷脂综合征(APS)是一种全身性自身免疫性疾病,其特点是存在抗磷脂抗体和静脉及动脉血栓事件,包括产科并发症。我们描述了一例被诊断为 APS 的 56 岁女性病例,她有三重抗体阳性和多种与疾病相关的表现,即复发性紫癜病变、梗死导致的肾上腺功能不全、结石性胆囊炎和三次自然流产。在她的随访过程中,发生了严重的血栓和出血事件,尤其是脾静脉血栓和肾活检后的失血性休克,以及在确诊 APS 8 年后被诊断为系统性红斑狼疮。在根据抗 Xa 因子活性决定用量的情况下,长期使用依诺肝素进行抗凝治疗,结果病情稳定了 17 年,没有出现并发症。本病例强调了个性化治疗策略和密切监测 APS 患者病情的重要性:该患者出现了抗磷脂综合征(APS)的罕见表现,如结石性胆囊炎、Budd-Chiari 综合征和 Addison 病,所有这些都与血管血栓形成引起的缺血性事件有关。在发生多次严重血栓和出血事件后,该患者成功接受了长期依诺肝素治疗,并根据抗因子 Xa 活性调整了剂量(与预测的经验剂量相比,可显著减少剂量)。
Complex Antiphospholipid Syndrome Successfully Controlled for 17 Years with Personalized Enoxaparin Therapy.
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the presence of antiphospholipid antibodies and venous and arterial thrombotic events, including obstetric complications. We describe the case of a 56-year-old female diagnosed with APS with triple antibody positivity and multiple disease-associated manifestations, namely recurrent purpuric lesions, adrenal insufficiency due to infarction, acalculous cholecystitis, and three spontaneous abortions. Her follow-up was marked by severe thrombotic and haemorrhagic events, notably splanchnic vein thrombosis and haemorrhagic shock after a renal biopsy, as well as the diagnosis of systemic lupus erythematosus 8 years after the APS diagnosis. Chronic anticoagulation with enoxaparin, with dosage guided by anti-factor Xa activity, resulted in stability without complications over 17 years. This case emphasizes the importance of personalized therapeutic strategies and close monitoring in patients with APS.
Learning points: This patient presented with rare manifestations of antiphospholipid syndrome (APS), such as acalculous cholecystitis, Budd-Chiari syndrome, and Addison's disease, all linked to ischemic events caused by vascular thrombosis. These atypical presentations highlight the varied and severe clinical spectrum of APS, requiring physicians to be vigilant about unusual complications.After multiple severe thrombotic and haemorrhagic events, the patient was successfully managed with long-term enoxaparin, with dose adjusted according to anti-factor Xa activity (which allowed for a significant dose reduction in comparison with the predicted empiric dose).The patient remained event-free for 17 years, reflecting the importance of personalizing anticoagulation therapy.
期刊介绍:
The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.