%0 Journal Article
%T Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature
%A B. Routy
%A T. Huynh
%A R. Fraser
%A C. S¨¦guin
%J Case Reports in Hematology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/710365
%X 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. 1. Introduction Antiphospholipid syndrome (APS) is characterized by the presence of antiphospholipid antibodies in patients who have a history of thrombosis and/or fetal loss. It is an autoimmune disease with a misleading name because the pathologic auto-antibodies are directed against the plasma protein ¦Â(2)-glycoprotein I and not against phospholipids [1]. Exceptionally, patients with APS may develop a ¡°catastrophic¡± variant [2]. Catastrophic Antiphospholipid Syndrome (CAPS) is defined as a life-threatening condition with widespread small vessel thromboses in a patient with laboratory confirmation of antiphospholipid antibodies [3]. First-line therapy includes a combination of anticoagulants, glucocorticoids, immunoglobulins, and plasma exchange [4¨C6]. Despite recent survival improvement related to the usage of newer immunosuppressive agents such as rituximab, CAPS still has an estimated 33.3% mortality rate [7]. Cardiac complications are the second most common cause of death after cerebral vascular disease [8¨C10]. The cardiac manifestations include valvular endocarditis and microvascular thromboses [9]. We report a case of a patient who developed fatal myocardial infarction and acute renal failure secondary to CAPS following an elective splenectomy. 2. Case Report A 79-year-old Indian man was referred to the Division of Hematology, McGill University Health Centre, Montreal, QC, Canada, for isolated thrombocytopenia. His past medical history was limited to mild dyslipidemia, and he was taking no medications. Physical examination was significant for the absence of hemorrhagic manifestations,
%U http://www.hindawi.com/journals/crihem/2013/710365/