E. Molinari et al., REFRACTORY THROMBOTIC THROMBOCYTOPENIC PURPURA - SUCCESSFUL TREATMENTBY PLASMAPHERESIS WITH PLASMA CRYOSUPERNATANT, Haematologica, 78(6), 1993, pp. 389-392
Background. Plasma exchange (PE) with reinfusion of fresh frozen plasm
a (FFP) is the therapy of choice for thrombotic thrombocytopenic purpu
ra (TTP) in the acute phase. About 20% of patients do not respond full
y to this treatment and can suffer relapse. The plasma cryosupernatant
(PCS) fraction depleted of the largest von Willebrand factor (VWF) mu
ltimers, considered to be among the possible causes of relapse, has re
cently been suggested as an alternative to FFP. Methods. We submitted
three patients in TTP relapse to plasma exchange with reinfusion of PC
S. This treatment was associated with anti-platelet agents in two of t
he patients. Results. Infusion of PCS led to a rapid improvement of th
e clinical picture in all three patients, with a return to normal of t
he reference parameters (platelet count' serum LDH). A few days after
suspending PE, the patient not receiving anti-platelet treatment suffe
red another relapse which was definitively resolved with resumption of
PE and administration of anti-platelet agents. Conclusions. We consid
er PCS to be a valid alternative treatment for TTP relapses, and we ha
ve found that the best results are obtained when it is associated with
anti-platelet agents.