Unresponsive Thrombotic Thrombocytopenic Purpura (TTP): Challenges and Solutions.

Therapeutics and Clinical Risk Management
Virginie LemialeEric Mariotte

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy secondary to a severely decreased A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats 13 (ADAMTS13) activity, resulting in the formation of widespread von Willebrand factor - and platelet-rich microthrombi. ADAMTS13 deficiency is mainly acquired through anti-ADAMTS13 autoantibodies in adults. With modern standards of care, unresponsive TTP has become rarer with a frequency of refractory/relapsing forms dropping from >40% to <10%. As patients with unresponsive TTP are at increased risk of mortality, prompt recognition and early therapeutic intensification are mandatory. Therapeutic options at the disposal of clinicians caring for patients with refractory TTP consist of increased ADAMTS13 supplementation, increased immunosuppression, and inhibition of von Willebrand factor adhesion to platelets. In this work, we focus on possible therapies for the management of patients with unresponsive TTP, and propose an algorithm for the management of these difficult cases.

References

Aug 22, 1992·Lancet·N T O'ConnorL F Hill
Nov 26, 1998·The New England Journal of Medicine·H M Tsai, E C Lian
May 31, 2001·European Journal of Clinical Investigation·G AnfossiM Trovati
Jan 17, 2003·Annals of Internal Medicine·Xinglong ZhengMorey A Blinder
Feb 18, 2003·British Journal of Haematology·Sarah L AllfordUNKNOWN Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology
Aug 24, 2005·British Journal of Haematology·M C Kappers-KlunneA Brand
Feb 15, 2007·Nature Clinical Practice. Rheumatology·Ronald P Taylor, Margaret A Lindorfer
Jul 22, 2008·British Journal of Haematology·Marie ScullySamuel Machin
Jan 27, 2011·The Journal of Clinical Investigation·Junmei ChenJosé A López
Feb 9, 2012·Seminars in Thrombosis and Hemostasis·Nancy TurnerJoel Moake
Mar 13, 2012·Transfusion·François BeloncleUNKNOWN Thrombotic Microangiopathies Reference Center
Mar 14, 2012·British Journal of Haematology·John ChapinJeffrey Laurence
Apr 25, 2012·Hematology·Marie Scully
Feb 26, 2013·British Journal of Clinical Pharmacology·Florent PuissetJacques Pourrat
Apr 4, 2013·Intensive Care Medicine·Eric MariotteElie Azoulay
Apr 27, 2013·Biochimica Et Biophysica Acta·Yuval SamuniMichael Berk
Dec 10, 2013·Hematology·James T B Crawley, Marie A Scully
May 30, 2014·Blood·Miguel HieUNKNOWN French Thrombotic Microangiopathies Reference Centre
Jul 22, 2014·Journal of Clinical Apheresis·Marleen M Neyrinck, Hans Vrielink
Aug 8, 2015·Journal of Thrombosis and Thrombolysis·Amihai RottenstreichYosef Kalish
Aug 22, 2015·European Journal of Internal Medicine·Antoine FroissartUNKNOWN French Reference Center for Thrombotic Microangiopathies
Feb 11, 2016·The New England Journal of Medicine·Flora PeyvandiUNKNOWN TITAN Investigators
Mar 25, 2016·British Journal of Haematology·Christopher J PatriquinMarie Scully
Nov 22, 2016·Journal of Thrombosis and Haemostasis : JTH·M ScullyUNKNOWN International Working Group for Thrombotic Thrombocytopenic Purpura
Jun 22, 2017·Mediators of Inflammation·Felice GragnanoPaolo Calabrò
Sep 13, 2017·Seminars in Thrombosis and Hemostasis·Paolo CalabròErik Lerkevang Grove
Oct 7, 2017·Advances in Therapy·Gilles SallesDavid G Maloney
Dec 17, 2017·American Journal of Hematology·Jennifer DingAnn B Zimrin

❮ Previous
Next ❯

Citations

Nov 9, 2021·Expert Review of Hematology·Katerina PavenskiChristopher J Patriquin

❮ Previous
Next ❯

Related Concepts

Related Feeds

Adhesion Molecules in Health and Disease

Cell adhesion molecules are a subset of cell adhesion proteins located on the cell surface involved in binding with other cells or with the extracellular matrix in the process called cell adhesion. In essence, cell adhesion molecules help cells stick to each other and to their surroundings. Cell adhesion is a crucial component in maintaining tissue structure and function. Discover the latest research on adhesion molecule and their role in health and disease here.

Related Papers

Blood
Bérangère S JolyAgnès Veyradier
[Rinshō ketsueki] The Japanese journal of clinical hematology
Masanori Matsumoto
Rinsho byori. The Japanese journal of clinical pathology
Seiji Kato, Yoshihiro Fujimura
[Rinshō ketsueki] The Japanese journal of clinical hematology
Masayuki Kubo, Masanori Matsumoto
© 2021 Meta ULC. All rights reserved