| Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. | |
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MedLine Citation:
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PMID: 11702904 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are multisystemic disorders that are characterized by thrombocytopenia, microangiopathic hemolytic anemia, and ischemic manifestations, resulting from platelet agglutination in the arterial microvasculature. Until the introduction of plasma-based therapy, TTP was associated with a mortality rate greater than 90%. Current outcomes of TTP and HUS have improved dramatically with the use of plasma exchange, which should be initiated promptly at diagnosis. Recent evidence suggests that deficiency of a specific plasma protease responsible for the physiologic degradation of von Willebrand factor plays a pathogenic role in a substantial proportion of familial and acute idiopathic cases of TTP. Although multiple triggers, such as infection, drugs, cancer, chemotherapy, bone marrow transplantation, and pregnancy, are recognized, knowledge of the pathogenesis of TTP and HUS in relationship to these disorders remains incompletely understood and continues to evolve. While uncommon, TTP and HUS are of considerable clinical importance because of their abrupt onset, fulminant clinical course, and high morbidity and mortality in the absence of early recognition and treatment. |
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Authors:
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M A Elliott; W L Nichols |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Mayo Clinic proceedings. Mayo Clinic Volume: 76 ISSN: 0025-6196 ISO Abbreviation: Mayo Clin. Proc. Publication Date: 2001 Nov |
Date Detail:
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Created Date: 2001-11-12 Completed Date: 2001-12-04 Revised Date: 2007-10-29 |
Medline Journal Info:
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Nlm Unique ID: 0405543 Medline TA: Mayo Clin Proc Country: United States |
Other Details:
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Languages: eng Pagination: 1154-62 Citation Subset: AIM; IM |
Affiliation:
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Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA. elliott.michelle@mayo.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Bone Marrow Transplantation Diagnosis, Differential Glucocorticoids / therapeutic use Hemolytic-Uremic Syndrome* / diagnosis, etiology, physiopathology, therapy Humans Plasma Exchange* Purpura, Thrombotic Thrombocytopenic* / diagnosis, physiopathology, therapy |
| Chemical | |
Reg. No./Substance:
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0/Glucocorticoids |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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