Abstract
The etiology of thrombotic thrombocytopenic purpura (TTP) remains undetermined. TTP has been associated with a number of secondary causes including infections, drugs, menses, pregnancy, autoimmune diseases, and bone marrow transplantation. Regardless of the inciting factors, it is widely accepted that endothelial injury and platelet aggregation are integral components. The morbidity and mortality have been significantly reduced with the use of plasmapheresis. However, refractory forms of TTP remain a clinical management challenge. Refractory TTP has not previously been associated with occult bacterial infection. Two patients had classic TTP that was refractory to daily plasma exchange with fresh-frozen plasma. Multiple attempts over a period of months to wean these patients off plasma exchange resulted in exacerbations of disease activity, as indicated by increased schistocytosis, decreased hematocrit, increased serum lactate dehydrogenase, and decreased platelet counts. Both patients were empirically treated for infections during hospitalization, although microbial cultures failed to isolate an organism. Discontinuation of antimicrobial therapy on multiple occasions in one patient was associated with recurrence of disease. In ...Continue Reading
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