BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a multisystem dise
ase characterized by the sudden onset of hemolytic anemia, thrombocytopenia
, fever, renal failure, and neurologic dysfunction, occurring in Various co
mbinations. The most effective treatment of TTP is plasma exchange and the
administration of corticosteroids, which reduces mortality from the 90 perc
ent seen in untreated patients to 10 percent.
CASE REPORTS: Two patients responded favorably to plasma exchange and corti
costeroid therapy, but their TTP relapsed during treatment. In both cases,
the early relapse of TTP was associated with Staphylococcus aureus bacterem
ia secondary to central line infection. Treatment of the infection and inte
nsification of the plasma exchange regimen resulted in a sustained remissio
CONCLUSION: Infection should be actively sought and treated in TTP patients
who are refractory to treatment or in whom an exacerbation of the disease
occurs while they are undergoing plasma exchange.