Jp. Hand et al., SUCCESSFUL USE OF CYCLOSPORINE-A IN THE TREATMENT OF REFRACTORY THROMBOTIC THROMBOCYTOPENIC PURPURA, British Journal of Haematology, 100(3), 1998, pp. 597-599
Thrombotic thrombocytopenic purpura (TTP) is, in most cases, treatable
with plasma exchange therapy (PLEX). Rarely, patients do not respond
to PLEX or develop refractory disease despite an initial remission. In
these cases, treatment options are limited and the response to establ
ished alternative therapies is often disappointing. We report the case
of a paediatric patient with TTP who developed refractory disease aft
er an initial response to PLEX. She was subsequently treated with cycl
osporine A and showed an immediate and sustained response. CSA may be
a safe and effective therapy for patients with refractory TTP and shou
ld be studied in randomized, prospective clinical trials.