Refractory thrombotic thrombocytopenic purpura: successful treatment by plasmapheresis with plasma cryosupernatant.

BACKGROUND

Plasma exchange (PE) with reinfusion of fresh frozen plasma (FFP) is the therapy of choice for thrombotic thrombocytopenic purpura (TTP) in the acute phase. About 20% of patients do not respond fully to this treatment and can suffer relapse. The plasma cryosupernatant (PCS) fraction depleted of the largest von Willebrand factor (vWF) multimers, considered to be among the possible causes of relapse, has recently been suggested as an alternative to FFP.

METHODS

We submitted three patients in TTP relapse to plasma exchange with reinfusion of PCS. This treatment was associated with anti-platelet agents in two of the patients.

RESULTS

Infusion of PCS led to a rapid improvement of the clinical picture in all three patients, with a return to normal of the reference parameters (platelet count' serum LDH). A few days after suspending PE, the patient not receiving anti-platelet treatment suffered another relapse which was definitively resolved with resumption of PE and administration of anti-platelet agents.

CONCLUSIONS

We consider PCS to be a valid alternative treatment for TTP relapses, and we have found that the best results are obtained when it is associated with anti-platelet agents.