Hu Xinxing, Liu Meihong
Department of Neurology, Loudi Central Hospital, Loudi, China.
Department of Oncology, Loudi Central Hospital, Loudi, China.
Medicine (Baltimore). 2024 Dec 13;103(50):e40829. doi: 10.1097/MD.0000000000040829.
Catastrophic antiphospholipid syndrome (CAPS) is the most serious type of antiphospholipid antibody syndrome (APS) and can be easily confused with other disorders, such as hemolytic uremic syndrome, disseminated intravascular coagulation and thrombocytopenia syndromes. Timely diagnosis of CAPS poses considerable challenges due to its rarity and the fact that clinicians often lack knowledge of the disease.
A 21-year-old patient was 32 weeks and 5 days pregnant when she presented to the hospital with a 7-hour history of sudden onset of left-sided limb weakness with no apparent cause. Lupus anticoagulant and/or anticardiolipin antibodies were positive. Head magnetic resonance imaging + magnetic resonance angiography + diffusion weighted imaging: right temporo-occipital insula, right basal ganglia and bilateral radial corona-hemispheric center showed multiple acute-phase cerebral infarction changes and right middle cerebral artery occlusion.
Catastrophic antiphospholipid syndrome.
By intracranial artery thrombectomy and anticoagulation with low-molecular heparin.
The patient's left limb muscle strength recovered to grade 5. A healthy baby boy was delivered by cesarean section. Both mother and child are safe.
The rarity of CAPS is such that misdiagnosis often occurs, culminating in serious complications and even death, emphasizing the need for early recognition, timely diagnosis and immediate treatment. In CAPS that improves with treatment, monitoring and prevention of recurrence is also essential.
灾难性抗磷脂综合征(CAPS)是抗磷脂抗体综合征(APS)最严重的类型,容易与其他疾病混淆,如溶血性尿毒症综合征、弥散性血管内凝血和血小板减少综合征。由于其罕见性以及临床医生通常对该疾病缺乏了解,CAPS的及时诊断面临相当大的挑战。
一名21岁患者,怀孕32周零5天时因左侧肢体突然无力7小时且无明显原因入院。狼疮抗凝物和/或抗心磷脂抗体呈阳性。头部磁共振成像+磁共振血管造影+弥散加权成像:右侧颞枕岛叶、右侧基底节及双侧放射冠-半卵圆中心多发急性期脑梗死改变,右侧大脑中动脉闭塞。
灾难性抗磷脂综合征。
通过颅内动脉取栓术及低分子肝素抗凝治疗。
患者左下肢肌力恢复至5级。剖宫产娩出一名健康男婴。母婴均安全。
CAPS的罕见性导致常发生误诊,最终引发严重并发症甚至死亡,强调早期识别、及时诊断及立即治疗的必要性。对于经治疗好转的CAPS患者,监测及预防复发也至关重要。