Rituximab for chronic recurring thrombotic thrombocytopenic purpura: A case report and review of the literature

Roslyn Yomtovian, Waldemar Niklinski, Bernard Silver, Ravindra Sarode, Han Mou Tsai

Research output: Contribution to journalReview articlepeer-review

112 Scopus citations

Abstract

Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 has been demonstrated to be the proximate cause of a subset of thrombotic microangiopathic haemolytic anaemias (MAHA) typical for thrombotic thrombocytopenic purpura (TTP). ADAMTS13 gene mutations cause the hereditary form; acquired deficiency has been attributed to presence of an autoantibody, which may represent a specific subset of MAHA best termed 'autoimmune thrombotic thrombocytopenic purpura'. We describe a patient with relapsing TTP because of ADAMTS13 inhibitors, who failed to achieve sustained remission despite therapies with plasma exchange, steroids, vincristine, staphylococcal protein A and splenectomy. The ADAMTS13 inhibitor titre remained elevated and clinical stability was only maintained by plasma exchange every 2-3 d over a period of 268 d. The patient then received rituximab therapy (eight doses of 375 mg/m2 weekly), during which she required five plasma exchanges in the first 10 d, two exchanges in the next 3 weeks, and none thereafter for 450 d and ongoing. The ADAMTS13 inhibitor titre decreased and enzyme activity increased. We compared this case with that of seven previously reported TTP cases also treated with rituximab; experience suggests that rituximab therapy deserves further investigation for patients with either refractory or relapsing TTP caused by ADAMTS13 inhibitors.

Original languageEnglish (US)
Pages (from-to)787-795
Number of pages9
JournalBritish Journal of Haematology
Volume124
Issue number6
DOIs
StatePublished - Mar 2004

Keywords

  • Plasma exchange
  • Rituximab
  • Thrombotic thrombocytopenic purpura

ASJC Scopus subject areas

  • Hematology

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