Hand J P, Lawlor E R, Yong C K, Davis J H
Department of Pediatrics, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada.
Br J Haematol. 1998 Mar;100(3):597-9. doi: 10.1046/j.1365-2141.1998.00602.x.
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 established alternative therapies is often disappointing. We report the case of a paediatric patient with TTP who developed refractory disease after an initial response to PLEX. She was subsequently treated with cyclosporine A and showed an immediate and sustained response. CSA may be a safe and effective therapy for patients with refractory TTP and should be studied in randomized, prospective clinical trials.
血栓性血小板减少性紫癜（TTP）在大多数情况下，可通过血浆置换疗法（PLEX）进行治疗。少数情况下，患者对PLEX无反应或尽管最初缓解但仍发展为难治性疾病。在这些病例中，治疗选择有限，对既定替代疗法的反应往往令人失望。我们报告了一例患有TTP的儿科患者，该患者在对PLEX最初有反应后发展为难治性疾病。她随后接受了环孢素A治疗，并显示出即时且持续的反应。环孢素A可能是治疗难治性TTP患者的一种安全有效的疗法，应在随机、前瞻性临床试验中进行研究。
