Routy B, Huynh T, Fraser R, Séguin C
Division of Haematology, Department of Medicine, McGill University Health Center (MUHC), 1650 Cedar Avenue, Montreal, QC, Canada H3G 1A4.
Case Rep Hematol. 2013;2013:710365. doi: 10.1155/2013/710365. Epub 2013 May 15.
Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.
灾难性抗磷脂综合征（CAPS）是一种罕见的自身免疫性疾病，与高死亡率相关。然而，通过目前包括抗凝、糖皮质激素给药和血浆置换相结合的治疗方法，死亡率已有所下降。尽管新一代免疫抑制剂提高了生存率，但其作用机制仍不清楚，CAPS在已知患有抗磷脂抗体综合征的患者中仍被认为是一种高风险并发症。在此，我们报告一例79岁男性患者，因免疫性血小板减少症行脾切除术后继发CAPS，出现心肌梗死和肾衰竭。尽管一线治疗与利妥昔单抗治疗迅速联合应用，但患者因二尖瓣乳头肌坏死继发致命性心源性休克。本文报道了对CAPS病理生理学及未来治疗途径的讨论。
