• Steffen Koschmieder, Wolfgang Miesbach, Florian Fauth, Joerg Bojunga, Inge Scharrer, and Hans-Reinhard Brodt.
• Zentrum für Innere Medizin, Universitätsklinikum, Johann Wolfgang Goethe-Universität, Frankfurt, Germany. skoschmi@caregroup.harvard.edu
• Blood Coagul. Fibrinolysis. 2003 Jun 1; 14 (4): 395-9.

AbstractWe report the case of a 33-year-old female with known primary antiphospholipid syndrome who, despite full-dose oral anticoagulation, presented with myocardial infarction, acute respiratory distress syndrome, purulent bronchitis, and septic shock. Antiphospholipid antibodies and anti-beta2-glycoprotein-1 titres were markedly elevated. The patient was diagnosed with catastrophic antiphospholipid syndrome and treated with unfractionated intravenous heparin. However, she developed thromboembolism of the right foot and skin marmoration of her extremities during heparin therapy, and therefore plasmapheresis, immunoglobulins, cyclophosphamide, and methylprednisone under a broad spectrum of anti-infective therapy were instituted. This treatment led to a rapid decrease of antiphospholipid antibody and anti-beta2-glycoprotein-1 titres, and the patient's condition gradually improved. Upon discharge from the hospital, pulmonary infiltrates had markedly regressed, and she was feeling well. Given the high mortality of catastrophic antiphospholipid syndrome, this report emphasizes the need for rapid diagnosis and effective multimodal treatment in an intensive care unit setting for these patients.

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