Thrombotic thrombocytopenic purpura
Top Cited Papers
- 6 April 2017
- journal article
- review article
- Published by Springer Science and Business Media LLC in Nature Reviews Disease Primers
- Vol. 3 (1), nrdp201720
- https://doi.org/10.1038/nrdp.2017.20
Abstract
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from ADAMTS13, whereas acquired TTP is an autoimmune disorder caused by circulating anti-ADAMTS13 autoantibodies, which inhibit the enzyme or increase its clearance. Consequently, immunosuppressive drugs, such as corticosteroids and often rituximab, supplement plasma exchange therapy in patients with acquired TTP.Keywords
This publication has 222 references indexed in Scilit:
- Ticlopidine‐associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR)British Journal of Haematology, 2013
- Modulation of the von Willebrand factor-dependent platelet adhesion through alternative proteolytic pathwaysThrombosis Research, 2012
- Generation and Breakdown of Soluble Ultralarge von Willebrand Factor MultimersSeminars in Thrombosis and Hemostasis, 2012
- Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factorProceedings of the National Academy of Sciences of the United States of America, 2009
- Blood donor satisfaction and intention of future donationTransfusion, 2008
- Hepatic stellate cell damage may lead to decreased plasma ADAMTS13 activity in ratsFEBS Letters, 2007
- Thrombotic Thrombocytopenic Purpura Associated with ClopidogrelNew England Journal of Medicine, 2000
- The role of splenectomy in the treatment of relapsing thrombotic thrombocytopenic purpuraAnnals of Hematology, 1995
- Unusually Large Plasma Factor VIII: von Willebrand Factor Multimers in Chronic Relapsing Thrombotic Thrombocytopenic PurpuraNew England Journal of Medicine, 1982
- Congenital Deficiency of a Factor in Normal Plasma That Reverses Microangiopathic Hemolysis and ThrombocytopeniaNew England Journal of Medicine, 1978