Although salvage therapy with rituximab is effective in some cases of immune-mediated thrombotic thrombocytopenic purpura (iTTP) refractory to standard plasma exchange (PEX) and glucocorticoid treatment or relapsed after treatment, protocols to address the subsequent high recurrence rate have not been established. We describe the use of cyclosporine (CSA) to prevent recurrence in a patient with iTTP relapse after rituximab therapy, and present a literature review. A 24-year-old woman was diagnosed with iTTP and initially received PEX and high-dose methylprednisolone therapy. However, weekly rituximab therapy was also needed for inhibitor boosting to achieve additional immunosuppression during the initial treatment. Although the patient achieved clinical remission after weekly rituximab therapy, iTTP relapsed twice when glucocorticoids were tapered, and was treated with a triplet regimen consisting of PEX, high-dose methylprednisolone, and weekly rituximab. CSA was administered along with glucocorticoids as prophylaxis against iTTP relapse. The additional CSA therapy successfully maintained iTTP remission and allowed reduction of the corticosteroid dose. Our findings demonstrate that prophylactic CSA can potentially prevent iTTP recurrence in patients with a history of multiple relapses. Data from more cases must be accumulated to establish a useful prophylactic therapy for iTTP that is refractory even to rituximab.
Keywords: Cyclosporine; Immune-mediated thrombotic thrombocytopenic purpura; Rituximab.
© 2022. Japanese Society of Hematology.