Document Detail

Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature.
MedLine Citation:
PMID:  15009067     Owner:  NLM     Status:  MEDLINE    
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.
Roslyn Yomtovian; Waldemar Niklinski; Bernard Silver; Ravindra Sarode; Han-Mou Tsai
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.; Review    
Journal Detail:
Title:  British journal of haematology     Volume:  124     ISSN:  0007-1048     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  2004 Mar 
Date Detail:
Created Date:  2004-03-10     Completed Date:  2004-05-20     Revised Date:  2014-09-18    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  England    
Other Details:
Languages:  eng     Pagination:  787-95     Citation Subset:  IM    
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MeSH Terms
ADAM Proteins
Antibodies, Monoclonal / therapeutic use*
Antibodies, Monoclonal, Murine-Derived
Chronic Disease
Combined Modality Therapy
Metalloendopeptidases / blood,  genetics
Plasma Exchange
Purpura, Thrombotic Thrombocytopenic / drug therapy*,  genetics
Grant Support
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Antibodies, Monoclonal, Murine-Derived; 0/rituximab; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases

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