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Platelets: Thrombotic Thrombocytopenic Purpura
| Content Provider | Scilit |
|---|---|
| Author | George, James N. Sadler, J. Evan Lämmle, Bernhard |
| Copyright Year | 2002 |
| Description | Abnormalities of plasma von Willebrand factor (VWF) have been recognized to be associated with thrombotic thrombocytopenic purpura (TTP) for over 20 years. Patients with chronic, relapsing TTP have VWF multimers that are larger than normal, similar in size to those secreted by cultured endothelial cells. Recent observations have documented that a deficiency of a VWF-cleaving protease (termed ADAMTS13) may be responsible for the presence of these unusually large VWF multimers. Multiple mutations of the ADAMTS13 gene can result in ADAMTS13 deficiency and cause congenital TTP; autoantibodies neutralizing ADAMTS13 protease activity have been associated with acquired TTP. |
| Related Links | http://asheducationbook.hematologylibrary.org/content/2002/1/315.full.pdf |
| Ending Page | 334 |
| Page Count | 20 |
| Starting Page | 315 |
| DOI | 10.1182/asheducation-2002.1.315 |
| Journal | Hematology |
| Issue Number | 1 |
| Volume Number | 2002 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 2002-01-01 |
| Access Restriction | Open |
| Subject Keyword | Hematology Von Willebrand Factor Journal: Hematology (Vol- 26, Issue- 1) |
| Content Type | Text |
| Resource Type | Article |
