灾难性抗磷脂综合征合并多重感染：病例报告
Catastrophic antiphospholipid syndrome complicated with multiple infections: a case report.
摘要(中文译文)
灾难性抗磷脂综合征（CAPS）是抗磷脂综合征（APS）一种罕见且危及生命的表现，常被延迟诊断且死亡率高。一名54岁女性，有网状青斑病史，因发热四天、血尿两天、意识障碍和瘀点一天就诊。实验室检查提示血小板减少、贫血、间接胆红素和乳酸脱氢酶水平升高，以及游离血红蛋白升高、直接抗球蛋白试验单纯C3d阳性、狼疮抗凝物和抗心磷脂IgG（aCL-IgG）阳性。诊断为自身免疫性溶血性贫血和血小板减少症，并开始给予大剂量甲泼尼龙联合静脉注射免疫球蛋白治疗。第4天，检出三尖瓣血栓，开始使用阿加曲班。尽管单纯LA阳性和低滴度aCL-IgG不符合经典APS标准，但明确的血栓形成使我们强烈怀疑准血清阴性APS，遂增加甲泼尼龙剂量。第6天，血小板计数再次下降，怀疑CAPS诊断，遂给予甲泼尼龙冲击治疗并联合血浆置换。第6天，血小板计数再次下降，患者接受甲泼尼龙冲击联合血浆置换治疗。第10天，出现白细胞减少并并发混合细菌感染。第11天，发生蛛网膜下腔出血，导致抗凝治疗中止。最终，患者因感染加重死亡。尸检显示广泛血栓形成和严重的播散性混合感染。CAPS的早期表现可能不典型，因此早期识别对治疗至关重要。在类固醇冲击治疗后，必须警惕真菌感染，必要时启动经验性抗真菌治疗。
Abstract
Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening manifestation of antiphospholipid syndrome (APS), which is frequently later diagnosed with high mortality. A 54-year-old woman with a history of livedo reticularis presented with fever for four days, hematuria for two days, and impaired consciousness and petechiae for one day. Laboratory tests indicated thrombocytopenia, anemia, elevated indirect bilirubin and lactate dehydrogenase levels, as well as elevated free hemoglobin, isolated C3d positivity on direct antiglobulin test, and positive lupus anticoagulant and anti-cardiolipin IgG (aCL-IgG). A diagnosis of autoimmune hemolytic anemia and thrombocytopenia was established, and high-dose methylprednisolone combined with intravenous immunoglobulin therapy was initiated. On day 4, a tricuspid valve thrombus was detected and argatroban was initiated. Although the isolated LA positivity and low-titer aCL-IgG did not fulfill classic APS criteria, the definitive thrombosis led us to strongly suspect quasi-seronegative APS, prompting an increased methylprednisolone dose. On day 6, the platelet count dropped again, and a diagnosis of CAPS was suspected, prompting the administration of methylprednisolone pulse therapy along with plasma exchange. On day 6, the platelet count dropped again, and the patient received methylprednisolone pulse along with plasma exchange treatment. On day 10, leukopenia complicated mixed bacterial infections were developed. On day 11, subarachnoid hemorrhage occurred, leading to discontinuation of anticoagulant. Ultimately, the patient died with the worsened infections. Autopsy revealed extensive thrombosis and severe disseminated mixed infections. Early manifestations of CAPS may be atypical, so early identification is crucial for treatment. Following steroid pulse therapy, vigilance for fungal infections is essential, with empirical antifungal therapy initiated when necessary.
如何引用
AMA
Lingli Zhou, Shuangshuang Liang, Huadong Zhu, Hua Chen, Jingjing Chai, Xiaojun Ma, et al. Catastrophic antiphospholipid syndrome complicated with multiple infections: a case report.. Frontiers in immunology. 2026; doi:10.3389/fimmu.2026.1764711.
APA
Lingli Zhou, Shuangshuang Liang, Huadong Zhu, Hua Chen, Jingjing Chai, Xiaojun Ma, et al (2026). Catastrophic antiphospholipid syndrome complicated with multiple infections: a case report.. Frontiers in immunology. https://doi.org/10.3389/fimmu.2026.1764711
GB/T 7714
Lingli Zhou, Shuangshuang Liang, Huadong Zhu, Hua Chen, Jingjing Chai, Xiaojun Ma, et al. Catastrophic antiphospholipid syndrome complicated with multiple infections: a case report.[J]. Frontiers in immunology, 2026 doi:10.3389/fimmu.2026.1764711.
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