Thrombotic thrombocytopenic purpura
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 (2024), 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.
- ↑ 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.