Maintenance treatment using the purine-synthesis inhibitor mizoribine in a patient with relapsing thrombotic thrombocytopenic purpura
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening disease. Although plasma exchange (PE) therapy and corticosteroids are standard remission induction and maintenance therapies, some patients are easily refractory and frequently relapse under treatment with this therapy, and require additional treatment. However, there are limited data about additional treatment interventions. We report a case of 56-year-old man who was hospitalized for fever, general fatigue and hemoglobinuria. Owing to the symptoms and the laboratory findings of hemolysis, he was diagnosed with TTP. He was treated with PE therapy and corticosteroids, and the TTP went into remission. However, his TTP relapsed and remission induction was attempted again. As a remission maintenance treatment, we used combination therapy with the purine-synthesis inhibitor mizoribine (MZR) and corticosteroids. The administration of MZR maintained disease activity with no adverse event for long periods and allowed us to gradually reduce the corticosteroids dose. Hence, we propose that MZR is an effective treatment for TTP maintenance.
KeywordsThrombotic thrombocytopenic purpura (TTP) Mizoribine (MZR) Hemoglobinuria
This work was supported by a Grant-in-Aid for Intractable Renal Diseases Research, Research on Rare and Intractable Diseases, Health and Labour Sciences Research Grants from the Ministry of Health, Labour and Welfare of Japan.
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Conflict of interest
All the authors have declared that no conflict of interest exists.
This article does not contain any studies with human participants performed by any of the authors.
No identifying information about individuals is included in the article.
- 10.Kaneko T, Hirama A, Ueda K, Fujino T, Utsumi K, Iino Y, et al. Methylprednisolone pulse therapy combined with mizoribine following tonsillectomy for immunoglobulin A nephropathy: clinical remission rate, steroid sparing effect, and maintenance of renal function. Clin Exp Nephrol. 2011;15:73 – 8.CrossRefPubMedGoogle Scholar