Vehbi DEMİRCAN, Abdullah KARAKUŞ, Mehmet Orhan AYYILDIZ
Journal of Current Hematology & Oncology Research - 2026;4(2):53-56
Aims : Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severe ADAMTS13 deficiency, leading to widespread microvascular thrombosis and requiring urgent therapeutic plasma exchange (TPE) and immunosuppression. Although most patients achieve remission with first-line TPE and corticosteroids, approximately 10-40% develop refractory disease requiring additional therapies. In our center, rituximab is administered in cases of plasma exchange and steroid failure. In the event of rituximab failure, vincristine is given as a third-line treatment. Methods : We conducted a retrospective single-center study of adult patients diagnosed with TTP between 2020 and 2025. All patients received first-line daily TPE plus corticosteroids. Refractoriness was defined as failure to achieve platelet recovery or biochemical improvement after 5-7 days of therapy. Patients laboratory results were obtained from the hospital's record system. Results : A total of 25 patients were included. First-line therapy achieved remission in 48% of patients (12/25). Among 13 refractory patients, rituximab induced remission in 2 (15%). Eleven patients subsequently received vincristine salvage therapy, of whom 9 (81.8%) achieved complete remission, accompanied by rapid platelet recovery and normalization of lactate dehydrogenase levels. Two elderly patients with severe multisystem involvement died despite salvage therapy. Overall survival for the cohort was 92%. Conclusion : Vincristine was highly effective and well tolerated as a salvage therapy in patients with refractory TTP who failed both TPE and rituximab, achieving remission in more than 80% of cases. These findings support vincristine as a valuable therapeutic option, particularly in settings where caplacizumab or other advanced biologics are unavailable. Larger prospective studies are warranted to better define its optimal timing and comparative efficacy among emerging salvage therapies.
