Management of thrombotic thrombocytopenic purpura

The first step in patient management is confirmation of the diagnosis. The clinical presentation must be considered with emphasis on the fact that the expression of a full pentad of symptoms is not required for diagnosis. A full blood count and blood smear should be reviewed and LDH and results of coagulation tests including PT, PTT and fibrinogen considered. If TTP is diagnosed or suspected, PE therapy should be commenced immediately. The 1.5 x plasma volume PE with FFP or CSP should be started with daily exchange for at least the first 3-4 d after presentation. Therapy should be maintained until the platelet count has risen to > 150 000 x 109/1 and has stabilized for at least 2 d. Long-term (months) PE should not be ruled out. Adjuvant therapy should also be used as considered warranted. There are no data indicating that antiplatelet agents have contributed to bleeding problems in these patients and they do appear to have benefit; the side-effects of steroid therapy are well known. In refractory patients, there appears to be no predictable pattern of response, therefore any of the adjuvant or alternative therapies including protein A immunoabsorption, vincristine and IVIgG should be considered. Until a definite aetiology and diagnostic test(s) are determined, practitioners will have to contend with the fact that although remarkable progress has been made in the treatment of TTP the treatment is still empirical and not yet predictably effective. Only by defining the true pathophysiology of this disorder will definitive therapy be possible. Certainly, much has been accomplished, but patients continue to die from this acute onset disorder involving platelet aggregate formation.