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Maintenance treatment using the purine-synthesis inhibitor mizoribine in a patient with relapsing thrombotic thrombocytopenic purpura

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Abstract

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.

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References

  1. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371:654–66.

    Article  CAS  PubMed  Google Scholar 

  2. George JN. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010;116:4060–9.

    Article  CAS  PubMed  Google Scholar 

  3. Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323–35.

    Article  PubMed  Google Scholar 

  4. Kremer Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115:1500–11 (quiz 662).

    Article  PubMed  Google Scholar 

  5. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura–hemolytic uremic syndrome. N Engl J Med. 1991;325:398–403.

    Article  CAS  PubMed  Google Scholar 

  6. Sayani FA, Abrams CS. How I treat refractory thrombotic thrombocytopenic purpura. Blood. 2015;125:3860–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Musa F, Baidas S. Idiopathic relapsing thrombotic thrombocytopenic purpura with persistent ADAMTS13 inhibitor activity treated sequentially with plasmapheresis, rituximab, cyclophosphamide and splenectomy. Case Rep Oncol. 2015;8:196–9.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Takahashi S, Wakui H, Gustafsson JA, Zilliacus J, Itoh H. Functional interaction of the immunosuppressant mizoribine with the 14-3-3 protein. Biochem Biophys Res Commun. 2000;274:87–92.

    Article  CAS  PubMed  Google Scholar 

  9. Igarashi Y, Moro Y, Kondo Y, Inoue CN. Steroid-sparing effect of mizoribine in long-term nephrotic syndrome of children. Pediatr Nephrol. 1994;8:396–7.

    Article  CAS  PubMed  Google Scholar 

  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.

    Article  CAS  PubMed  Google Scholar 

  11. Nakayamada S, Saito K, Umehara H, Ogawa N, Sumida T, Ito S, et al. Efficacy and safety of mizoribine for the treatment of Sjogren’s syndrome: a multicenter open-label clinical trial. Mod Rheumatol. 2007;17:464–9.

    Article  CAS  PubMed  Google Scholar 

  12. Yamashita H, Takahashi Y, Kaneko H, Kano T, Mimori A. Thrombotic thrombocytopenic purpura with an autoantibody to ADAMTS13 complicating Sjogren’s syndrome: two cases and a literature review. Mod Rheumatol. 2013;23:365–73.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

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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Authors and Affiliations

  1. Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan

    Tetsuya Kawamura, Joichi Usui, Kei Nagai & Kunihiro Yamagata

  2. Department of Nephrology, St. Mary’s Hospital, Kurume, Fukuoka, Japan

    Kentaro Sakai

  3. Department of Nephrology, Mito Saiseikai General Hospital, Mito, Ibaraki, Japan

    Itaru Ebihara

Authors
  1. Tetsuya Kawamura
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  2. Joichi Usui
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  3. Kei Nagai
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  4. Kentaro Sakai
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  5. Itaru Ebihara
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  6. Kunihiro Yamagata
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Correspondence to Joichi Usui.

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Kawamura, T., Usui, J., Nagai, K. et al. Maintenance treatment using the purine-synthesis inhibitor mizoribine in a patient with relapsing thrombotic thrombocytopenic purpura. CEN Case Rep 7, 24–28 (2018). https://doi.org/10.1007/s13730-017-0285-y

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  • DOI: https://doi.org/10.1007/s13730-017-0285-y

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