Study of Cyclosporine or Corticosteroids as an Adjunct to Plasma Exchange in Thrombotic Thrombocytopenic Purpura (TTP)

Inclusion Criteria:

• Patients with a clinical diagnosis of idiopathic TTP as defined by a microangiopathichemolytic anemia and thrombocytopenia (<100 x 103)
• Additional components of the pentad (fever, renal and neurologic abnormalities) neednot be present.
• Additional explanations for the microangiopathic changes including DIC and malignancyshould be excluded.
• Patients with pregnancy associated TTP will be permitted on this therapeutic trial ifthe child is delivered prior to the initiation of therapy for TTP. However, femalepatients that are breastfeeding and are unwilling to discontinue breastfeeding at thetime of enrollment will be excluded from this study
• Patients with a previous diagnosis of TTP are eligible to be enrolled provided theymeet eligibility criteria and have not been treated for an TTP in the past 30 days
• Given the potential for nephrotoxicity with CSA, all patients must have a serumcreatinine of < 2.5 mg/dl prior to enrollment

Exclusion Criteria:

• In light of concern for the prompt initiation of PE, all patients with suspected TTPmay be enrolled on this trial. If it is subsequently found that the patient does notmeet enrollment criteria, they will be removed and their spot replaced for studypurposes. Patients removed from the study after enrollment will continue to befollowed longitudinally for 6 months to be monitored for safety and will be includedin the safety database.
• Patients with TTP clinically categorized as secondary to stem cell transplant andsolid organ, bloody diarrhea associated, malignancy associated, and drug associatedwill not be enrolled on this therapeutic study.
• Incarcerated patients will be excluded from the study due to the inherent difficultiesin maintaining close follow-up for study purposes in patients who are incarcerated.
• Any patients already being treated chronically with corticosteroids or cyclosporineand taking these at the time of their presentation will be excluded from this study.
• Female patients that are breastfeeding and are unwilling to discontinue breastfeedingat the time of enrollment will be excluded from this study
• Patients taking any medications contraindicated in combination with CSA that cannot besafely discontinued will be excluded from this study.