Journal ArticleDOI
Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura.
Gail Rock,Kenneth H. Shumak,Noel A. Buskard,Victor S. Blanchette,John G. Kelton,Rama C. Nair,Robert A. Spasoff +6 more
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TLDR
A prospective randomized trial comparing plasma exchange with plasma infusion for the treatment of thrombotic throm bocytopenic purpura finds plasma exchange is more effective than plasma infusion.Abstract:
Background. Thrombotic thrombocytopenic purpura is an uncommon disease with a high mortality rate even with current treatment. The cause of the syndrome and its optimal treatment are unknown. Although both plasma exchange and plasma infusion have been useful treatments, it is not clear which is superior. In this report we describe a prospective randomized trial comparing plasma exchange with plasma infusion for the treatment of thrombotic thrombocytopenic purpura. Methods. One hundred two patients with thrombotic thrombocytopenic purpura were randomly assigned to receive either plasma exchange or plasma infusion with fresh-frozen plasma on seven of the first nine days after entry into the trial. The total volume of plasma received by patients undergoing plasma exchange was three times that received by patients undergoing plasma infusion. All the patients also received aspirin and dipyridamole. The outcomes in the two groups were compared at the end of the first treatment cycle (day 9) and after s...read more
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Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue
Joseph E. Schwartz,Jeffrey L. Winters,Anand Padmanabhan,Rasheed A. Balogun,Meghan Delaney,Michael L. Linenberger,Zbigniew M. Szczepiorkowski,Mark E. Williams,Yanyun Wu,Beth H. Shaz +9 more
TL;DR: The Eighth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.
Journal ArticleDOI
Antibodies to von willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura
Han-Mou Tsai,Eric Chun-Yet Lian +1 more
TL;DR: Inhibitory antibodies against von Willebrand factor-cleaving protease occur in patients with acute thrombotic throm bocytopenic purpura, suggesting that a deficiency of this protease is likely to have a critical role in the pathogenesis of platelet thromBosis in this disease.
Journal ArticleDOI
Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura
Gallia Levy,William C. Nichols,Eric C.-Y. Lian,Tatiana Foroud,Jeanette N. McClintick,Beth McGee,Angela Y. Yang,David R. Siemieniak,Kenneth R. Stark,Ralph A. Gruppo,Ravindra Sarode,Susan B. Shurin,Visalam Chandrasekaran,Sally P. Stabler,Hernan Sabio,Eric E. Bouhassira,Jefferson D. Upshaw,David Ginsburg,Han-Mou Tsai +18 more
TL;DR: In this article, the ADAMTS family of zinc metalloproteinase genes (ADAMTS13) was identified as the molecular mechanism responsible for TTP, and it was shown that the deficiency of ADADTS13 is the molecular mechanisms responsible for the development of TTP.
Journal ArticleDOI
Syndromes of Thrombotic Microangiopathy
James N. George,Carla M. Nester +1 more
TL;DR: This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.
Journal ArticleDOI
Structure of von Willebrand factor cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura
Xinglong Zheng,Dominic W. Chung,Thomas K. Takayama,Elaine M. Majerus,J. Evan Sadler,Kazuo Fujikawa +5 more
TL;DR: The 4.6-kilobase pair cDNA sequence for VWFCP has been determined and may have functional significance, producing proteins with distinct abilities to interact with cofactors, connective tissue, platelets, and von Willebrand factor.
References
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TL;DR: In this article, a generalization of the Kruskal-Wallis test for testing the equality of K continuous distribution functions when observations are subject to arbitrary right censorship is proposed, where the distribution of the censoring variables is allowed to differ for different populations.
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Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature
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Journal ArticleDOI
Treatment of thrombotic thrombocytopenic purpura with plasma.
John J. Byrnes,Mohan Khurana +1 more
TL;DR: Since the pathogenesis of this disease is not understood, treatment has been empirical or with an assumed rationale, among the various modes of therapy tried, four are especially advocated: splenectomy and use of antiplatelet agents, of high doses of glucocorticoids, and of exchange transfusions.
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