Refractory thrombotic thrombocytopenic purpura in a 16-year-old girl: successful treatment with bortezomib
SourceEuropean Journal of Haematology, 92, 1, (2014), pp. 80-82
Article / Letter to editor
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Paediatrics - OUD tm 2017
European Journal of Haematology
SubjectRadboudumc 11: Renal disorders RIMLS: Radboud Institute for Molecular Life Sciences
We present a case of a 16-year-old girl with autoimmune thrombotic thrombocytopenic purpura (TTP), refractory to plasma exchange and high-dose prednisone. Despite the additional treatment with rituximab, she developed renal and neurological complications with ongoing hemolysis and thrombocytopenia. Bortezomib, a proteasome inhibitor and thereby blocking plasma cells, was added, and our patient recovered. We suggest that bortezomib can be of additional value in severe immunologically mediated TTP in adolescents. Its use may prevent the necessity of other invasive therapies, such as splenectomy, with significant side effects.
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