Thrombotic thrombocytopenic purpura: Difference between revisions

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==Diagnosis==
==Diagnosis==
In the Oklahoma registry, 23% (60/261) patients with idiopathic thrombotic thrombocytopenic purpura had ADAMTS13 activity of less than 10%.<ref name="pmid20032506">{{cite journal| author=Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN| title=Survival and relapse in patients with thrombotic thrombocytopenic purpura. | journal=Blood | year= 2010 | volume= 115 | issue= 8 | pages= 1500-11; quiz 1662 | pmid=20032506 | doi=10.1182/blood-2009-09-243790 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20032506  }} </ref><ref name="pmid12637323">{{cite journal| author=Vesely SK, George JN, Lämmle B, Studt JD, Alberio L, El-Harake MA et al.| title=ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. | journal=Blood | year= 2003 | volume= 102 | issue= 1 | pages= 60-8 | pmid=12637323 | doi=10.1182/blood-2003-01-0193 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12637323  }} </ref>
In the Oklahoma registry, 23% (60/261) patients with idiopathic thrombotic thrombocytopenic purpura had ADAMTS13 activity of less than 10%.<ref name="pmid20032506"/><ref name="pmid12637323">{{cite journal| author=Vesely SK, George JN, Lämmle B, Studt JD, Alberio L, El-Harake MA et al.| title=ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. | journal=Blood | year= 2003 | volume= 102 | issue= 1 | pages= 60-8 | pmid=12637323 | doi=10.1182/blood-2003-01-0193 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12637323  }} </ref>


==References==
==References==
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Latest revision as of 14:31, 9 October 2024

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In medicine and hematology, thrombotic thrombocytopenic purpura (TTP) is "an acquired, congenital, or familial disorder caused by platelet aggregation with thrombosis in terminal arterioles and capillaries. Clinical features include thrombocytopenia; hemolytic anemia; azotemia; fever; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as renal failure."[1]

Etiology/cause

Patients with primary TTP may have low von Willebrand factor-degrading protease (ADAMTS13 protein), an ADAM Protein.[2] This deficiency allows thrombi due to von Willebrand Factor (vWF)

Diagnosis

In the Oklahoma registry, 23% (60/261) patients with idiopathic thrombotic thrombocytopenic purpura had ADAMTS13 activity of less than 10%.[2][3]

References