دورية أكاديمية

Effectiveness of Caplacizumab Nanobody in Acquired Thrombotic Thrombocytopenic Purpura Refractory to Conventional Treatment.

التفاصيل البيبلوغرافية
العنوان: Effectiveness of Caplacizumab Nanobody in Acquired Thrombotic Thrombocytopenic Purpura Refractory to Conventional Treatment.
المؤلفون: Palanques-Pastor, Tomás, Megías-Vericat, Juan Eduardo, Bosó Ribelles, Virginia, Gómez Seguí, Inés, Poveda Andrés, José Luis
المصدر: Acta Haematologica; Jan2022, Vol. 145 Issue 1, p72-77, 6p
مصطلحات موضوعية: THROMBOTIC thrombocytopenic purpura, THROMBOPENIC purpura, VON Willebrand factor, PLATELET count, THROMBOSPONDIN-1
مستخلص: Acquired thrombocytopenic thrombotic purpura (aTTP) is an autoantibody-mediated disease against the enzyme A Disintegrin and Metalloprotease domain with ThromboSpondin-1 type motif 13, which until now has been treated with plasma exchange (PEX) and corticosteroids. A 29-year-old female patient, who presented with aTTP in the context of pregnancy, has developed multiple relapses after treatment with PEX, corticosteroids, and rituximab. Recently, caplacizumab, a nanobody against von Willebrand factor, has been approved for the treatment of aTTP. In our patient, caplacizumab achieved better disease control, with a lower platelet count restoration time, days of PEX and hospitalization duration, as compared to standard therapy, reproducing the results of clinical trials. Caplacizumab represents a significant advance in the treatment of aTTP, especially in cases of recurrent relapses. [ABSTRACT FROM AUTHOR]
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قاعدة البيانات: Complementary Index
الوصف
تدمد:00015792
DOI:10.1159/000517813