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- Title
Effectiveness of Caplacizumab Nanobody in Acquired Thrombotic Thrombocytopenic Purpura Refractory to Conventional Treatment.
- Authors
Palanques-Pastor, Tomás; Megías-Vericat, Juan Eduardo; Bosó Ribelles, Virginia; Gómez Seguí, Inés; Poveda Andrés, José Luis
- Abstract
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.
- Subjects
THROMBOTIC thrombocytopenic purpura; THROMBOPENIC purpura; VON Willebrand factor; PLATELET count; THROMBOSPONDIN-1
- Publication
Acta Haematologica, 2022, Vol 145, Issue 1, p72
- ISSN
0001-5792
- Publication type
Article
- DOI
10.1159/000517813