Thrombotic thrombocytopenic purpura: Difference between revisions
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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]]."<ref>{{MeSH}}</ref> | 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]]."<ref>{{MeSH}}</ref> | ||
Patients with primary TTP may have low von Willebrand factor-degrading protease (ADAMTS13 protein), an [[ADAM Protein]].<ref name="pmid20032506">{{cite journal| author=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 | ==Etiology/cause== | ||
| url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&retmode=ref&cmd=prlinks&id=20032506 | doi=10.1182/blood-2009-09-243790 }} </ref> This deficiency allows thrombi due to von Willebrand | Patients with primary TTP may have low von Willebrand factor-degrading protease (ADAMTS13 protein), an [[ADAM Protein]].<ref name="pmid20032506">{{cite journal| author=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 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=clinical.uthscsa.edu/cite&retmode=ref&cmd=prlinks&id=20032506 | doi=10.1182/blood-2009-09-243790 }} </ref> 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%.<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> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} |
Revision as of 20:03, 25 September 2013
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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
- ↑ Anonymous (2025), Thrombotic thrombocytopenic purpura (English). Medical Subject Headings. U.S. National Library of Medicine.
- ↑ 2.0 2.1 Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN (2010). "Survival and relapse in patients with thrombotic thrombocytopenic purpura.". Blood 115 (8): 1500-11; quiz 1662. DOI:10.1182/blood-2009-09-243790. PMID 20032506. Research Blogging.
Cite error: Invalid
<ref>
tag; name "pmid20032506" defined multiple times with different content - ↑ Vesely SK, George JN, Lämmle B, Studt JD, Alberio L, El-Harake MA et al. (2003). "ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients.". Blood 102 (1): 60-8. DOI:10.1182/blood-2003-01-0193. PMID 12637323. Research Blogging.