Singh Som, Candiales Sergio Abraham, Hunzeker Zachary, Freites Cristina Olivo, Idowu Modupe
Division of Hematology, Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, USA.
AME Case Rep. 2025 Jul 15;9:79. doi: 10.21037/acr-24-190. eCollection 2025.
There are few cases that report the presence of lupus anticoagulant and antiphospholipid antibodies between Evans syndrome and the presence of lupus anticoagulant. However, differentiating between primary and secondary causes of Evans syndrome remains challenging.
A 31-year-old female with a medical history of autism spectrum disorder, antiphospholipid syndrome (APS), complete systemic lupus erythematosus (SLE), and Evans syndrome was admitted for cholecystitis requiring laparoscopic cholecystectomy. In the setting of her acute illness, her thrombocytopenia worsened. Her hospital course was then complicated by the development of a left adrenal hematoma and venous thrombosis. Anticoagulation therapy was conservatively held due to concern for bleeding risk with the hematoma. However, given persistent thrombocytopenia, she subsequently underwent a platelet transfusion and was started on a 5-day course of intravenous immunoglobulin (IVIG). A venous Doppler ultrasound revealed a new occlusive deep venous thrombosis in the right axillary and brachial veins and occlusive venous thrombosis in the left cephalic veins while on the conservative anticoagulation regimen. This led to the immediate restart of anticoagulation therapy with close monitoring of coagulation labs. Her platelets improved Eltrombopag and IVIG up to 50,000 platelets/mcL.
This case aims to highlight the challenges of managing thrombocytopenia in a patient with multiple autoimmune conditions on opposite ends of the coagulopathic spectrum in the inpatient setting.
很少有病例报告Evans综合征与狼疮抗凝物之间存在狼疮抗凝物和抗磷脂抗体。然而,区分Evans综合征的原发性和继发性病因仍然具有挑战性。
一名31岁女性,有自闭症谱系障碍、抗磷脂综合征(APS)、完全性系统性红斑狼疮(SLE)和Evans综合征病史,因胆囊炎入院,需要进行腹腔镜胆囊切除术。在她急性发病期间,血小板减少症加重。随后,她的病程因左肾上腺血肿和静脉血栓形成而复杂化。由于担心血肿有出血风险,抗凝治疗被保守地暂停。然而,鉴于持续的血小板减少症,她随后接受了血小板输注,并开始了为期5天的静脉注射免疫球蛋白(IVIG)疗程。在保守抗凝方案治疗期间,静脉多普勒超声显示右腋窝和肱静脉出现新的闭塞性深静脉血栓,左头静脉出现闭塞性静脉血栓。这导致立即重新开始抗凝治疗,并密切监测凝血实验室指标。她的血小板计数通过艾曲泊帕和IVIG提高到了50,000/微升。
本病例旨在强调在住院环境中,管理一名患有多种自身免疫性疾病且处于凝血病变谱两端的患者的血小板减少症所面临的挑战。