期刊
EUROPEAN JOURNAL OF HAEMATOLOGY
卷 92, 期 1, 页码 80-82出版社
WILEY-BLACKWELL
DOI: 10.1111/ejh.12206
关键词
thrombotic thrombocytopenic purpura; bortezomib; rituximab; plasma exchange; ADAMTS13 autoantibodies
类别
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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