Shoaib Khan Muhammad, Ishaq Muhammad, Siorek Marek, Biederman Robert
Department of Internal Medicine, Marshfield Clinic Health System, Marshfield, Wisconsin, USA.
Department of Interventional Radiology, Marshfield Clinic Health System, Marshfield, Wisconsin, USA.
Caspian J Intern Med. 2021;12(Suppl 2):S487-S490. doi: 10.22088/cjim.12.0.487.
Antiphospholipid syndrome (APS) is marked by arterial, venous, or small vessel thrombosis. There have been few reported cases on APS presenting as thrombosis simultaneously involving large arteries and venous side of the blood circulation. CAPS can easily be confused with DIC, HIT, and other TMA. Anticoagulants remain the mainstay of treatment for CAPS, whereas in DIC and TMA, anticoagulants have no role.
A 43-year-old male presented to our facility with a chief complaint of right foot pain, calf cramps, and shortness of breath. The patient's right dorsal pedal artery was not palpable. CT angiogram showed bilateral pulmonary emboli (fig.1), emboli within the right saphenofemoral artery (SFA), and popliteal artery (PA). Digital subtraction angiogram showed occlusive thrombi in SFA and in the PA. Thrombolysis was performed by an intra-arterial catheter-directed tissue plasminogen activator. Agitated saline bubble study showed no evidence of atrial shunting. The patient was noted to have thrombocytopenia, hypofibrinogenemia, high serum D-Dimer and normal activated partial thromboplastin time (APTT). The patient tested positive for anticardiolipin (aCL) antibodies and lupus anticoagulant (LA). After 12 weeks, aCL antibodies and LA testing were suggestive of APS.
Simultaneous thrombosis in large arteries and veins is a very unusual presentation for the APS. The patients should be started on anticoagulants immediately as the mortality rate associated with CAPS is high and the key to management is initiating anticoagulants expeditiously.
抗磷脂综合征(APS)以动脉、静脉或小血管血栓形成为特征。关于APS表现为血栓形成同时累及体循环的大动脉和静脉侧的报道病例很少。灾难性抗磷脂综合征(CAPS)很容易与弥散性血管内凝血(DIC)、肝素诱导的血小板减少症(HIT)及其他血栓性微血管病(TMA)相混淆。抗凝剂仍是CAPS治疗的主要手段,而在DIC和TMA中,抗凝剂并无作用。
一名43岁男性因主诉右脚疼痛、小腿痉挛和呼吸急促前来我院就诊。患者右足背动脉搏动未触及。CT血管造影显示双侧肺栓塞(图1)、右股隐静脉(SFA)和腘动脉(PA)内有栓子。数字减影血管造影显示SFA和PA内有闭塞性血栓。通过动脉内导管定向组织纤溶酶原激活剂进行溶栓治疗。振荡生理盐水气泡试验未显示心房分流的证据。该患者出现血小板减少、纤维蛋白原血症、血清D-二聚体升高和活化部分凝血活酶时间(APTT)正常。该患者抗心磷脂(aCL)抗体和狼疮抗凝物(LA)检测呈阳性。12周后,aCL抗体和LA检测提示为APS。
大动脉和静脉同时发生血栓形成是APS非常罕见的表现。由于与CAPS相关的死亡率很高,且治疗的关键是迅速启动抗凝治疗,因此应立即开始对患者进行抗凝治疗。