Oredegbe Al-Ameen, Robledo Fabiana Maria Santelises, Hongalgi Krishnakumar, Faddoul Geovani, Mehta Swati
Albany Medical Center, Albany, New York, USA.
Albany Medical Center, Albany, New York, USA.
Am J Med Sci. 2023 Jan;365(1):104-108. doi: 10.1016/j.amjms.2022.08.010. Epub 2022 Aug 27.
Catastrophic anti-phospholipid syndrome (CAPS) is characterized by microvascular thrombosis in multiple sites leading to multi-organ damage. It is a rare and fatal complication of antiphospholipid syndrome (APS). We present a rare case of CAPS that presented with bilateral (b/l) adrenal hemorrhage making the diagnosis challenging in this otherwise rare disease. A 51-year-old female was initially admitted with abdominal pain and found to have bilateral adrenal hemorrhage. Patient had a fulminant disease course in which she had thrombotic manifestations involving multiple organ systems. This case was especially challenging as the patient's bilateral adrenal hemorrhage was the first manifestation of CAPS; the diagnosis of APS had to be made while treatment for presumed CAPS was emergently commenced for this life-threatening disease. Key to managing this condition is having a high index of suspicion for the diagnosis in patients presenting with multi-organ failure and multiple thromboses and hemorrhage.
灾难性抗磷脂综合征(CAPS)的特征是多部位微血管血栓形成,导致多器官损伤。它是抗磷脂综合征(APS)一种罕见且致命的并发症。我们报告了一例罕见的CAPS病例,该病例表现为双侧肾上腺出血,这使得在这种本就罕见的疾病中进行诊断具有挑战性。一名51岁女性最初因腹痛入院,被发现有双侧肾上腺出血。患者病情急骤,出现累及多个器官系统的血栓形成表现。该病例尤其具有挑战性,因为患者的双侧肾上腺出血是CAPS的首发表现;在针对这种危及生命的疾病紧急开始假定的CAPS治疗时,必须做出APS的诊断。管理这种疾病的关键是对出现多器官功能衰竭、多处血栓形成和出血的患者高度怀疑该诊断。