Until recently no specific treatment was available
and mortality rates were very high.
The introduction of plasma
exchange (PE) and other
treatments led to successful management in the majority of
cases.
PE can only be performed in specialized transfusion
centres and consists of the exchange of patient's plasma with
healthy donor plasma during one or more sessions. Good results
have also been obtained in genetic TTP cases with infusion
of fresh frozen plasma of healthy donors.
These procedures should be associated with immune
suppression therapy (corticosteroids, vincristine,
ciclophosphamide and other drugs) or therapy with drugs
that inhibit platelets' aggregates.
Recently rituximab,
a drug previously used to treat lymphomas and other autoimmune
diseases, has been reported to induce longstanding remissions
in patients affected by autoimmune
TTP who suffered from multiple
recurrences, whose disease was partially or not responsive
to other immunosuppressive agents. Therapy with rituximab
is still sperimental and only a few patients are eligible
for treatment.
