THROMBOTIC MICROANGIOPATHIC HEMOLYTIC-ANEMIA IN SYSTEMIC LUPUS-ERYTHEMATOSUS

Thrombotic microangiopathic hemolytic anemia (TMHA) is characterized b y thrombocytopenia, microangiopathic hemolytic anemia, fever, neurolog ical symtoms, and kidney involvement. It presents as thrombotic thromb ocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS). TMHA has been considered to occur only rarely in systemic lupus erythematosus ( SLE). However, there has been an increase in the reporting of this ass ociation in recent years, and autopsy studies have suggested that TMHA may be underdiagnosed in SLE because of the similarity in symptoms. W e report four patients with SLE-related TMHA and describe 24 more pati ents from a literature review. All patients were women, 50% had active SLE, 89% presented as TTP, and 11% presented as HUS. Those patients w ith active SLE had low complement levels. Antiphospholipid antibodies or lupus anticoagulant were positive in 5 of 8 cases. Patients treated with plasma infusions or plasmapheresis had a lower mortality rate at 25% compared with 57% mortality in patients who were not treated with plasma infusions or plasmapheresis. It is suggested that TMHA should be considered in any SLE patient presenting with neurological symptoms or renal failure associated with fever, hemolytic anemia, and thrombo cytopenia. Early recognition and appropriate therapy with plasmapheres is may improve prognosis. Copyright (C) 1994 by W.B. Saunders Company