Recent Advances in Thrombotic Thrombocytopenic Purpura
Open Access
- 1 January 2004
- journal article
- review article
- Published by American Society of Hematology in Hematology-American Society Hematology Education Program
- Vol. 2004 (1) , 407-423
- https://doi.org/10.1182/asheducation-2004.1.407
Abstract
Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. Intravascular coagulation is not a prominent feature of the disorder. Plasma exchange can induce remissions in approximately 80% of patients with idiopathic TTP, but patients have a much worse prognosis when thrombotic microangiopathy is associated with cancer, certain drugs, infections, or tissue transplantation. Recently, acquired autoimmune deficiency of a plasma metalloprotease named ADAMTS13 was shown to cause many cases of idiopathic TTP. This review describes our current understanding of how to use this knowledge clinically.Keywords
This publication has 61 references indexed in Scilit:
- ADAMTS-13 Metalloprotease Interacts with the Endothelial Cell-derived Ultra-large von Willebrand FactorJournal of Biological Chemistry, 2003
- ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditionsBlood, 2002
- Thrombotic MicroangiopathiesNew England Journal of Medicine, 2002
- Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: studies using optical tweezersBlood, 2002
- Structure of von Willebrand Factor-cleaving Protease (ADAMTS13), a Metalloprotease Involved in Thrombotic Thrombocytopenic PurpuraJournal of Biological Chemistry, 2001
- Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpuraNature, 2001
- Antibodies to von Willebrand Factor–Cleaving Protease in Acute Thrombotic Thrombocytopenic PurpuraNew England Journal of Medicine, 1998
- von Willebrand Factor–Cleaving Protease in Thrombotic Thrombocytopenic Purpura and the Hemolytic–Uremic SyndromeNew England Journal of Medicine, 1998
- Involvement of large plasma von Willebrand factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation.Journal of Clinical Investigation, 1986
- Unusually Large Plasma Factor VIII: von Willebrand Factor Multimers in Chronic Relapsing Thrombotic Thrombocytopenic PurpuraNew England Journal of Medicine, 1982