ISSN:
1437-7799
Keywords:
systemic lupus erythematosus
;
microangiopathic hemolytic anemia
;
thrombotic thrombocytopenic purpura
;
plasmapheresis
;
acute renal failure
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract We report on a 15-year-old girl who had systemic lupus erythematosus (SLE), hemolytic anemia, thrombocytopenia, acute renal failure, and central nervous system disorder including an episode of convulsions. Red cell fragmentation was observed in the peripheral blood, but only a slight abnormality was noted in the coagulative/fibrinolytic system. She was treated with corticosteroid pulse therapy, hemodialysis, and plasmapheresis and anticoagulants. The severe anemia and thrombocytopenia responded to immunoadsorption. The renal biopsy specimen showed a thickening of the small arteries with a narrowing of the lumens, and an immunofluorescent study revealed deposits of fibrinogen in the renal blood vessels. A provisional diagnosis of SLE with thrombotic thrombocytopenic purpura was made. A magnetic resonance image of the brain obtained at the time of a convulsion showed multiple, scattered high-intensity areas on the rim of the cerebral cortex; these disappeared after treatment. These findings were strikingly similar to those observed in central nervous system lupus. We describe a case of SLE with microangiopathic hemolytic anemia that provides an insight into the mechanisms of thrombotic thrombocytopenic purpura and SLE that affect the central nervous system.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF02480700
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