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Bortezomib for chronic relapsing thrombotic thrombocytopenic purpura: a case report
- Source :
- Transfusion. 54(8)
- Publication Year :
- 2013
-
Abstract
- Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterized by a severe deficiency of ADAMTS13 activity. Although therapeutic plasma exchange (PLEX) is the standard of care, 30% to 50% patients develop exacerbation or relapse, requiring immunomodulatory agents. Of these agents, glucocorticoids, rituximab, and cyclosporine A are the most frequently used.We report a case of chronic relapsing TTP in a patient who had eight relapses over a 14-year period. After her seventh relapse, the patient demonstrated only partial response to glucocorticoids, two courses of rituximab, and cyclophosphamide. The eighth relapse occurred 58 days after her last PLEX and subsequent to this she received a course of bortezomib (Velcade, Millennium Pharmaceuticals, Inc.). After treatment with bortezomib the patient demonstrated a complete response with a progressive increase in ADAMTS13 activity from less than 5% to 22% accompanied by undetectable inhibitor, and she has remained PLEX free for more than 169 days.Bortezomib may serve as an adjunct treatment in patients with acquired TTP who exhibit an incomplete response or are refractory to conventional management.
- Subjects :
- Purpura, Thrombotic Thrombocytopenic
Remission Induction
Drug Resistance
ADAMTS13 Protein
Middle Aged
Staphylococcal Infections
Boronic Acids
Bortezomib
ADAM Proteins
Antibodies, Monoclonal, Murine-Derived
Immunocompromised Host
Recurrence
Pyrazines
Humans
Female
Rituximab
Cyclophosphamide
Glucocorticoids
Immunosuppressive Agents
Subjects
Details
- ISSN :
- 15372995
- Volume :
- 54
- Issue :
- 8
- Database :
- OpenAIRE
- Journal :
- Transfusion
- Accession number :
- edsair.pmid..........4762c9d2ee2a5ebac9b4106fd8b11e8a