Treatment of thrombotic thrombocytopenic purpura

Vox Sanguinis
Stefano FontanaB Mansouri Taleghani

Abstract

Thrombotic thrombocytopenic purpura (TTP), characterized by thrombocytopenia and microangiopathic haemolytic anaemia, was almost universally fatal until the introduction of plasma exchange (PE) therapy in the 1970s. Based on clinical studies, daily PE has become the first-choice therapy since 1991. Recent findings may explain its effectiveness, which may include, in particular, the removal of anti-ADAMTS13 autoantibodies and unusually large von Willebrand factor multimers and/or supply of ADAMTS13 in acquired idiopathic or congenital TTP. Based on currently available data, the favoured PE regimen is daily PE [involving replacement of 1-1.5 times the patient's plasma volume with fresh-frozen plasma (FFP)] until remission. Adverse events of treatment are mainly related to central venous catheters. The potential reduction of plasma related side-effects, such as transfusion-related acute lung injury (TRALI) or febrile transfusion reactions by use of solvent-detergent treated (S/D) plasma instead of FFP is not established by controlled clinical studies. Uncontrolled clinical observations and the hypothesis of an autoimmune process in a significant part of the patients with acquired idiopathic TTP suggest a beneficial effect of adjun...Continue Reading

References

Jul 1, 1966·Naika. Internal medicine·Y SugaM Nomura
Jun 1, 1994·British Journal of Haematology·N NovitzkyW Rosenstrauch
Mar 3, 1998·Transfusion·H G KleinE L Snyder
Jun 10, 1998·Annals of Internal Medicine·C L BennettD Green
Oct 28, 1998·Vox Sanguinis·E BaudouxD Sondag-Thull
Nov 26, 1998·The New England Journal of Medicine·H M Tsai, E C Lian
Jan 4, 2001·Journal of Cardiothoracic and Vascular Anesthesia·A D SharmaH P Grocott
Feb 13, 2001·British Journal of Haematology·S L AllfordS J Machin
Apr 20, 2001·Journal of Clinical Apheresis·Z R ZeiglerUNKNOWN North American TTP Group
May 1, 2001·British Journal of Haematology·M F MurphyUNKNOWN British Committee for Standards in Haematology, Blood Transfusion Task Force
Aug 23, 2002·The New England Journal of Medicine·Joel L Moake
Oct 16, 2002·Thrombosis Research·Peter HellsternBjarte G Solheim
Nov 9, 2002·Transfusion·Javier de la RubiaMaría L Marty
Feb 18, 2003·British Journal of Haematology·Sarah L AllfordUNKNOWN Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology
Aug 28, 2003·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·G RockUNKNOWN CAAN

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Citations

Dec 15, 2010·Journal of Artificial Organs : the Official Journal of the Japanese Society for Artificial Organs·Sanjeev BawejaLawrence P McMahon
Oct 23, 2008·International Journal of Hematology·Nana SasakiMasafumi Taniwaki
Jan 9, 2010·International Journal of Hematology·Joseph E Kiss
May 12, 2007·Critical Care : the Official Journal of the Critical Care Forum·Spyros A PapirisCharis Roussos
Jan 29, 2014·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Yuki KojimaHirokazu Nagai
Oct 24, 2009·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Len VerbekeDaan Dierickx
Mar 27, 2009·The American Journal of the Medical Sciences·Muralikrishna GopalJack B Alperin
Nov 21, 2009·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Gurhan KadikoyluZahit Bolaman
May 20, 2008·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Jerard SeghatchianStefan Reichenberg
Nov 2, 2007·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·H JalankoT S Jokiranta
Jun 27, 2006·Journal of Thrombosis and Haemostasis : JTH·T Matsushita, H Saito
Jun 26, 2012·Journal of Clinical Apheresis·Somchai YongsiriNatthaphon Annanon
May 18, 2010·Pediatric Hematology and Oncology·Somasundaram JayaboseClaudio Sandoval
Feb 2, 2008·Pediatric Blood & Cancer·Hayley BornRobert Ettinger
Jul 7, 2020·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·A D MakatsariyaT Mashkova
Aug 22, 2018·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Santhosh Kumar DevadasNavin Khattry
Sep 18, 2018·Memo·Paul Knöbl
Feb 2, 2021·International Immunopharmacology·Hamed Azhdari TehraniShirin Haghighi

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