Combination vincristine and plasma exchange as initial therapy in patients with thrombotic thrombocytopenic purpura: one institution's experience and review of the literature.

摘要

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) was once a highly fatal disease with mortality reaching nearly 95 percent; however, application of therapeutic plasma exchange (TPE) has dramatically increased survival. Nevertheless, mortality remains substantial (10%-30% in many published reports), requiring the search for more efficacious treatments. Vincristine (VCR) has been generally reserved for refractory TTP. Despite its effectiveness in a salvage mode, VCR has not been widely advocated as first-line therapy in conjunction with TPE. We previously reported improved survival when VCR and TPE were administered at presentation in patients treated from 1979 to 1994. Utilizing this standardized approach, outcomes of an additional group of patients and the results of a literature review of VCR therapy for TTP are reported. STUDY DESIGN AND METHODS: Medical records of all patients with a diagnosis of TTP treated between 1995 and 2002 at Cedars-Sinai Medical Center were reviewed. TPE was performed daily, exchanging 1.25 plasma volumes, until the platelet count normalized. Patients received VCR 1.4 mg/m2, (up to 2.0 mg total dose) after the first TPE. A literature review of all publications utilizing VCR in the management of TTP was performed with MEDLINE. RESULTS: Twelve consecutive patients meeting the diagnostic criteria received treatment with VCR and TPE. All patients achieved durable remission. Patients tolerated VCR without significant complications. CONCLUSION: Our 100 percent survival rate, as well as evidence garnered from the literature review, suggests that combination therapy with VCR and TPE at presentation might be more effective than TPE alone and therefore warrants consideration as first-line therapy for TTP patients.