Therapeutic plasma exchange in the treatment of the hereditary thrombotic thrombocytopenic purpura

BACKGROUND: Therapeutic plasma exchange is the treatment of choice for thrombotic thrombocytopenic purpura (TTP). METHODS AND RESULTS: Patients chronically treated with plasma exchange are frequently exposed to a large number of single plasma donors units, however successful clinical and laboratory improvement is generally achieved. Therapeutic plasma exchange significantly decreased mortality of this disease. Plasma treatment offers two possibilities--plasma infusion or plasma exchange and possibility of different plasma types. Cryoprecipitate-poor plasma was introduced as better form of FFP, however studies presented later did not confirm the therapeutic benefit. Quarantine FFP is widely used for patients with TTP in the Czech Republic; this type is tested frequently for negativity of human immunodeficiency virus, hepatitis B virus, hepatitis C virus and syphilis. Treatment with pathogen inactivated plasma (e.g., solvent detergent plasma, methylen-blue plasma, psoralen treated plasma or riboflavin treated plasma) is not frequently used in the Czech Republic. Authors present different plasma types and their experience with plasma treatment in seven patients with congenital form of TTP. CONCLUSIONS: In five patients therapeutic plasma exchange with quarantine fresh frozen plasma uas used. During each treatment 1.5 of plasma volume was exchanged. Two patients--teenagers were treated at the ex paediatric clinic with plasma infusion in regularly 2 weeks intervals.