Document Detail


Untying the knot of thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome.
MedLine Citation:
PMID:  23410558     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Patients presenting with microangiopathic hemolysis and thrombocytopenia are often given the diagnosis of thrombotic thrombocytopenic purpura and treated with plasma exchange until the acute episode is over. Recent findings have shown that acquired thrombotic thrombocytopenic purpura is a chronic autoimmune disease with inhibitory antibodies of a disintegrin and metalloprotease with thrombospondin repeat, member 13 and are at risk of relapses that may be preventable. Furthermore, many of the patients given the diagnosis of thrombotic thrombocytopenic purpura really have atypical hemolytic uremic syndrome due to defective complement regulation that can be more effectively treated to prevent death and end-stage renal failure with eculizumab, a humanized monoclonal antibody of complement C5. These advances indicate that an accurate differential diagnosis of microangiopathic hemolysis is essential for optimal patient management.
Authors:
Han-Mou Tsai
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of medicine     Volume:  126     ISSN:  1555-7162     ISO Abbreviation:  Am. J. Med.     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-02-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0267200     Medline TA:  Am J Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  200-9     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2013 Elsevier Inc. All rights reserved.
Affiliation:
iMAH Hematology Associates, New Hyde Park, NY. Electronic address: hmtsai@gmail.com.
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