Vnitr Lek 2025, 71(7):462-467 | DOI: 10.36290/vnl.2025.083

Our first experience with caplacizumab in TTP

Eva Drbohlavová, Kristýna Datinská
Oddělení klinické hematologie Krajské nemocnice Liberec, a. s.

Thrombotic thrombocytopenic purpura (TTP) is a rare disease with high mortality. Its prognosis depends on early diagnosis and timely initiation of treatment when there is reasonable suspicion of this disease. Until 2021, the gold standard was a combination of immunosuppression and therapeutic plasma exchange (TPE) (1,2). Caplacizumab is now available as a first-line treatment, significantly changing the rate of therapeutic response in patients with acquired TTP (3-5). The case of our patient demonstrates how early initiation of treatment with caplacizumab, together with immunosuppression and TPE, brought her back to life.

Keywords: ADAMTS13, caplacizumab, immunosuppression, therapeutic plasma exchange, thrombotic thrombocytopenic purpura.

Accepted: October 16, 2025; Published: October 31, 2025  Show citation

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Drbohlavová E, Datinská K. Our first experience with caplacizumab in TTP. Vnitr Lek. 2025;71(7):462-467. doi: 10.36290/vnl.2025.083.
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    References

    1. Lánská M, Radocha J. Kaplacizumab v léčbě získané trombotické trombocytopenické purpury. Acta Med. 2022;4.
    2. Lánská M. Léčba získané trombotické trombocytopenické purpury - kazuistika. Farmakoterapeutické Revue. 2022;7(6).
    3. Scully M, Cataland SR, Peyvandi F, et al. Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura. N Engl J Med. 2019;380(4):335346. Go to original source... Go to PubMed...
    4. Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura. N Engl J Med. 2016;374(6):511522. Go to original source... Go to PubMed...
    5. Elverdi T, Eskazan AE. Caplacizumab as an emerging treatment option for acquired thrombotic thrombocytopenic purpura. Drug Des Devel Ther [Internet]. 2019;13:12511258. Available from: doi:10.2147/DDDT.S134470. Go to original source... Go to PubMed...
    6. Zheng XL, Vesely SK, Cataland SR, Coppo P, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):24862495. Go to original source... Go to PubMed...
    7. Cablivi. SPC.
    8. Knoebl P, Cataland S, Peyvandi F, et al. Efficacy and safety of openlabel caplacizumab in patients with exacerbations of acquired thrombotic thrombocytopenic purpura in the HERCULES study. J Thromb Haemost. 2020;18(2):479484. Go to original source... Go to PubMed...
    9. Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2017;15(7):14481452. Go to original source... Go to PubMed...
    10. Wali JA, Quigley BM, Schaefer B. Successful Use of Bortezomib in an Adolescent with Refractory TTP. Case Rep Hematol. 2023;2023:8173903. Go to original source... Go to PubMed...
    11. Lee CJN, Yates S, Rambally S, Sarode R, et al. Bortezomib in relapsed/refractory immune thrombotic thrombocytopenic purpura: a singlecentre retrospective cohort and systematic literature review. Br J Haematol [Internet]. 2023:??? Available from: https://doi.org/10.1111/bjh.19035. Go to original source... Go to PubMed...
    12. Yin J, Tian H, Kong D, et al. Bortezomib, a promising alternative for patients with refractory or relapsed thrombotic thrombocytopenic purpura after rituximab treatment. Br J Haematol [Internet]. 2022. Available from: https://doi.org/10.1111/bjh.18430. Go to original source... Go to PubMed...
    13. Weisinger J, Fadlallah J, Joly B, et al. How we manage immunemediated thrombotic thrombocytopenic purpura after rituximab failure or intolerance. Br J Haematol. 2025;in press. Go to original source... Go to PubMed...
    14. Long X, AlHousni Z, Cataland SR, et al. 2025 focused update of the 2020 ISTH Guidelines for management of thrombotic thrombocytopenic purpura. ISTH SSC Commun [Internet]. 2025:in press.
    15. Cuker A, Cataland SR, Coppo P, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021;137(14):18551861. Go to original source... Go to PubMed...

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