Catastrophic antiphospholipid syndrome. Two forms of presentation.

Antiphospholipid syndrome is characterized by recurrent fetal loss, arteria l and venous thromboses, thrombocytopenia and circulating antiphospholipid antibodies. Few patients have a rapidly progressive, fatal outcome. We repo rt two young patients with systemic lupus erythematosus and antiphospholipi d antibodies who died after a short course of disease. Although clinical an d laboratory findings differed in both patients -small vessel thromboses an d microangiopathic hemolytic anemia mimicking thrombotic thrombocytopenic p urpura predominated in one of the patients while small and medium size vess el thromboses without hemolysis were present in the other case- autopsy rev ealed widespread visceral thromboses in both of them, features consistent w ith a diagnosis of catastrophic antiphospholipid syndrome. This syndrome ha s not been reported to occur in association with Pneumocistis carinii pneum onia as we describe in one of our patients.