C. Mazzei et al., TREATMENT OF THROMBOTIC THROMBOCYTOPENIC PURPURA - A ROLE FOR EARLY VINCRISTINE ADMINISTRATION, Journal of clinical apheresis, 13(1), 1998, pp. 20-22
Plasma exchange (PE) is considered first-line treatment for thrombotic
thrombocytopenic purpura (TTP) to the point that many clinicians rega
rd it as definitive therapy. Studies have reported response rates to P
E ranging from 39% to 78%. In our experience, a minority of patients h
ave been cured solely by PE. While adjuvant therapies (e.g., vincristi
ne, splenectomy) have proved effective in anecdotal reports, protocols
using these therapies in the treatment of TTP have not been establish
ed. Management of TTP over a 15-year period was reviewed to evaluate (
1) the rate of cure accomplished by PE alone, and (2) the potential be
nefit of additional therapies. The records of 29 consecutive patients
with TTP treated by PE were reviewed and classified according to respo
nse to PE alone and the need for adjuvant therapy. Eight patients (28%
) achieved remission and long-term survival with PE alone. With the ad
dition of adjuvant therapy another 13 patients survived, bringing the
total survival to 72%. Fifteen patients were treated with vincristine
in addition to PE. Only three of seven patients receiving vincristine
after failing to respond completely to PE survived, but survival incre
ased to 88% (7 of 8) when vincristine was administered within 3 days o
f beginning PE. These data suggest that PE alone may not be sufficient
therapy for most patients with ?TP. Additional therapy is often neede
d to achieve long-term survival. While controlled trials will be neces
sary to prove the efficacy of vincristine, we believe that, given the
minimal risk of vincristine toxicity and the grave consequences of ine
ffective therapy, routine administration of vincristine early in the c
ourse of PE should be considered. (C) 1998 Wiley-Liss, Inc.