Membranous obstruction of the inferior vena cava and extensive bone marrow necrosis associated with catastrophic antiphospholipid syndrome.
The catastrophic antiphospholipid syndrome is a rare and devastating manifestation of the antiphospholipid syndrome. It is associated with acute multi-organ failure involving three or more end organs in patients with antiphospholipid antibodies. A precipitating event can be found in some cases, and mortality rates have approached 60%. This article describes a case of catastrophic antiphospholipid syndrome in a patient who presented with Budd-Chiari syndrome from membranous obstruction of the inferior vena cava and extensive bone marrow necrosis shortly after a surgical procedure. These and other clinical manifestations of multi-organ failure (including low-grade disseminated intravascular coagulation) were associated with high titers of immunoglobulin M anticardiolipin antibodies. The patient fully recovered after being treated with percutaneous transluminal angioplasty and aggressive anticoagulation. The pathogenesis of catastrophic antiphospholipid syndrome in this patient may well have resulted from in situ thrombus formation around an intravascular coagulation web (congenital or acquired) from a patient predisposed to a hypercoagulable state from anticardiolipin antibodies and a recent surgical procedure.
Spyropoulos
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Department of Internal Medicine, Medical Director Anticoagulation Clinic, Lovelace Health Systems, Albuquerque, New Mexico.