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Microangiopathic haemolytic anaemia resembling thrombotic thrombocytopenic purpura in systemic lupus erythematosus: the role of ADAMTS13.
MedLine Citation:
PMID:  21149242     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Thrombotic thrombocytopenic purpura (TTP) is a rare but frequently fatal complication of SLE. It occurs in the context of both active and inactive lupus and carries a worse overall prognosis than idiopathic acquired TTP. Recent advances in the knowledge and treatment of TTP do not seem to have brought similar improvements in the management and outcome of TTP in SLE. The illumination of the role of the von Willebrand factor multimer protease, ADAMTS13 in idiopathic TTP continues to enhance our comprehension of the pathogenesis of the disease and has contributed to improvements in diagnosis and management. We explore the overlap of TTP and SLE, and discuss the current understanding of the involvement of ADAMTS13 and its implications for patients with this uncommon form of microangiopathic haemolytic anaemia.
Authors:
Frederick Lansigan; Iris Isufi; Clement E Tagoe
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2010-12-11
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  -     ISSN:  1462-0332     ISO Abbreviation:  -     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Hematology/Oncology, Dartmouth-Hitchcock Medical Center, Lebanon, NH, Department of Medical Oncology, Yale University School of Medicine, New Haven, CT and Department of Medicine, Albert Einstein College of Medicine, Bronx, NY, USA.
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