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Therapeutic Challenges with Catastrophic Antiphospholipid Antibody Syndrome: A Case Report

机译:灾难性抗磷脂抗体综合征的治疗挑战:案例报告

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Antiphospholipid antibody syndrome (APS) is a multisystem disorder characterized by thromboembolic events in the presence of antiphospholipid antibodies (APLA). Catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon variant of APS?which is associated with widespread coagulopathy that predominantly affects small vessels. Despite maximal treatment, CAPS has a very high?mortality rate. We present a case of a 42-year-old woman with a history of APS who presented to our hospital with complaints of epistaxis, hemoptysis, menorrhagia, and shortness of breath. She was diagnosed with CAPS and developed multiorgan failure and sepsis. Despite maximal treatment with immune modulators, she unfortunately succumbed. With this case, we highlight the importance of early recognition of CAPS and review various treatment modalities that have been proven beneficial. Despite these modalities, CAPS remains a therapeutic challenge in many cases and has a high mortality rate.
机译:抗磷脂抗体综合征(APS)是在抗磷脂抗体(APLA)存在下以血栓栓塞事件为特征的多系统障碍。灾难性的抗磷脂抗体综合征综合征(帽)是AP的罕见变体?这与广泛的凝血病相关,主要是影响小血管。尽管有最大的处理,帽子具有非常高的?死亡率。我们提出了一个42岁女性的案例,拥有历史的APS,他们向我们医院提出了existaxis,咯血,月经和呼吸急促。她被诊断出患有CAPS并开发了多性衰竭和败血症。尽管用免疫调节剂处理了最大的治疗,但她不幸的是屈服了。在这种情况下,我们强调了早期识别帽的重要性,并审查已被证明有益的各种治疗方式。尽管这些方式,但在许多情况下,CAP仍然是治疗性挑战,并且死亡率很高。

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