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Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature

Authors

  • Murvet Yilmaz,

    Corresponding author
    1. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    2. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    3. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
    • Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
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  • Ahmet Emre Eskazan,

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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  • Abdulkadir Unsal,

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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  • Hakan Taninmis,

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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  • Ekrem Kara,

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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  • Mustafa Cetiner,

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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  • Burhan Ferhanoglu

    1. Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey
    2. Department of Hematology, Diyarbakir Training and Research Hospital, Diyarbakir, Turkey
    3. Department of Nephrology, Sisli Etfal Teaching Hospital, Istanbul, Turkey
    4. Division of Hematology, Department of Internal Medicine, VKV American Hospital, Istanbul, Turkey
    5. Division of Hematology, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey
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Address reprint requests to: Murvet Yilmaz, Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Bakirkoy, Istanbul 34303, Turkey; e-mail: murvetyilmaz@hotmail.com.

Abstract

Background

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disturbances, and renal failure. Plasma therapy has dramatically improved prognosis of TTP, whereas recurrent acute episodes still occur in approximately 40% of patients. Moreover, patients with acquired ADAMTS13 deficiency, which is a significant factor for relapse, may require additional immunosuppressive treatment to get a durable remission.

Study Design and Methods

We hereby report two patients with a history of relapsed idiopathic TTP, who both received cyclosporin A (CSA) as a prophylactic manner after the remission was achieved. We also discuss the efficacy of CSA in patients with relapsed idiopathic TTP with a review of the published literature.

Results

Under CSA therapy, both patients maintained their clinical remission state, and the ADAMTS13 levels were normalized.

Conclusion

To conclude, CSA therapy may be useful for the prevention of relapsed idiopathic TTP in patients with a history of frequent relapses.

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