Bortezomib for chronic relapsing thrombotic thrombocytopenic purpura: A case report

Sean Yates, Karen Matevosyan, Cynthia Rutherford, Yu Min Shen, Ravi Sarode

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Background 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. Case Report 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. Conclusion Bortezomib may serve as an adjunct treatment in patients with acquired TTP who exhibit an incomplete response or are refractory to conventional management.

Original languageEnglish (US)
Pages (from-to)2064-2067
Number of pages4
Issue number8
StatePublished - Aug 2014

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Hematology


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