Journal articleJ Thromb Thrombolysis · October 2025
Acute limb ischemia (ALI) is characterized by a sudden decrease in limb perfusion due to arterial occlusion. Without urgent revascularization, patients are at risk of ischemic damage and amputation. This study uses novel proteomic techniques to investigate ...
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Journal articleBlood Adv · January 28, 2025
Immune thrombotic thrombocytopenic purpura (iTTP) is a chronically relapsing disorder caused by autoantibody-mediated deficiency of ADAMTS13. Rituximab is frequently administered to prevent relapses, but whether the durability of rituximab effect is mainta ...
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Journal articleBlood · September 12, 2024
In the 100 years since Eli Moschcowitz reported the first case of thrombotic thrombocytopenic purpura (TTP), there has been remarkable awareness and progress in the diagnosis and management of this rare blood disorder. This progress initially was the resul ...
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Journal articleResearch and Practice in Thrombosis and Haemostasis · March 1, 2024
Background: Mortality due to immune-mediated thrombotic thrombocytopenic purpura (iTTP) remains significant. Predicting mortality risk may potentially help individualize treatment. The French Thrombotic Microangiopathy (TMA) Reference Score has not been ex ...
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Journal articleHaemophilia · September 2023
INTRODUCTION: Recombinant porcine factor VIII (rpFVIII, susoctocog alfa) is indicated for the treatment of bleeding episodes in adults with acquired haemophilia A (AHA). AIM: To provide long-term real-world safety and effectiveness data for rpFVIII in the ...
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Journal articleJ Thromb Haemost · December 2022
INTRODUCTION: Caplacizumab demonstrated efficacy and safety in patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP) in the phase 3 HERCULES trial. However, data on long-term outcomes following caplacizumab treatment are limited. OBJECTI ...
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Journal articleBlood · September 22, 2022
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is characterized by recurring episodes of thrombotic microangiopathy, causing ischemic organ impairment. Black patients are overrepresented in iTTP cohorts in the United States, but racial disparit ...
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Journal articleTransfusion · February 2022
BackgroundThis study investigated the real-world safety and tolerability of solvent/detergent-treated (S/D) plasma for pediatric patients requiring therapeutic plasma exchange (TPE).Study design and methodsLAS-213 was a multicenter, open- ...
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Journal articleBlood Adv · April 27, 2021
The efficacy and safety of caplacizumab in individuals with acquired thrombotic thrombocytopenic purpura (aTTP) have been established in the phase 2 TITAN and phase 3 HERCULES trials. Integrated analysis of data from both trials was conducted to increase s ...
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Journal articleClin Immunol · December 2020
In complement-driven thrombotic microangiopathies, failure to regulate complement activation leads to end-organ damage. The modified Ham (mHam) test measures complement-mediated killing of a nucleated cell in vitro but lacks a confirmatory assay and reliab ...
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Journal articleJ Thromb Haemost · February 2020
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Caplacizumab, an anti-von Willebrand Factor Nanobody® , is effective for treating aTTP episodes and is well tolerated. OBJECT ...
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Journal articleN Engl J Med · January 24, 2019
BACKGROUND: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which ...
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Journal articleJ Clin Apher · August 2018
INTRODUCTION: Limited data are available describing indications for and outcomes of therapeutic plasma exchange (TPE) in cardiac transplantation. METHODS: In a retrospective study of patients who underwent cardiac transplantation at Duke University Medical ...
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Journal articleJ Thromb Thrombolysis · April 2018
Essential thrombocythemia (ET) is an indolent myeloproliferative neoplasm characterized by megakaryocyte hyperplasia, thrombocytosis, thrombotic and hemorrhagic complications, and potential transformation into myelofibrosis and acute myeloid leukemia. The ...
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Journal articleCurr Opin Hematol · November 2017
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with t ...
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Journal articleTher Apher Dial · October 2017
The human leukocyte antigen (HLA) system is an important immunologic barrier that must be considered for successful solid organ transplantation. Formation of donor-specific HLA antibodies in solid organ transplantation is an important cause of allograft in ...
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Journal articleJ Clin Apher · October 2016
The thrombotic microangiopathy (TMA) Registry Network of North America (TRNA) is a collaborative network organized for the purpose of developing a multi-institutional registry and network to conduct clinical studies in a rare patient population. The TRNA w ...
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Journal articleBlood · October 22, 2015
In this issue of Blood, Gill et al describe the results of the first phase 3 clinical trial evaluating recombinant von Willebrand factor (rVWF) for the treatment of hemorrhagic events in all patients with von Willebrand disease (VWD). ...
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Journal articleCase Rep Oncol Med · 2014
Background. Recent literature has associated hepatitis C virus with the development of non-Hodgkin lymphoma. Hepatitis C virus infection appears to promote lymphoproliferation, providing a plausible mechanism for a causative association; however, despite p ...
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Journal articleObstet Gynecol · August 2013
BACKGROUND: Romiplostim, a thrombopoietin mimetic, is a novel therapeutic option for patients with chronic immune thrombocytopenic purpura. We report on the effects of romiplostim use throughout pregnancy. CASE: A 28-year-old primigravid woman with chronic ...
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Journal articleHaemophilia · July 2013
Type 3 von Willebrand's disease (VWD) is a rare bleeding diathesis with complete or near complete deficiency of von Willebrand factor (VWF) and low factor VIII (FVIII) levels. In contrast, only FVIII is decreased in haemophilia A (HA). Both disorders are c ...
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Journal articleBlood · September 9, 2010
The diagnosis of heparin-induced thrombocytopenia (HIT) requires detection of antibodies to the heparin/platelet factor 4 (PF4) complexes via enzyme-linked immunosorbent assay. Addition of excess heparin to the sample decreases the optical density by 50% o ...
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Journal articleHaemophilia · July 2009
Type 3 von Willebrand disease (VWD) is a rare bleeding disorder with markedly decreased or absent von Willebrand factor (VWF) protein, accompanied by a parallel decrease in VWF function and factor VIII (FVIII) activity. The goal of this study was to descri ...
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Journal articleBiologics · 2009
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Decades of basic science and clinical research have led to an increased understanding of the pathophysiology of immune thrombocytopenic purpura (ITP), the processes underlying thrombopoiesis, and the treatment of chronic ITP. Now, new agents are available ...
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Journal articleHematology Am Soc Hematol Educ Program · 2009
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A 35-year-old female presents with a prolonged activated partial thromboplastin time (aPTT) on routine testing, which is found to be due to a lupus anticoagulant. She has no medical issues, no personal or family history of thrombosis, no history of pregnan ...
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Journal articleExpert Rev Cardiovasc Ther · January 2008
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In recent years, great advances have been made in elucidating the pathogenesis of thrombocytopenia and the mechanisms of thrombopoiesis. Drawing upon decades of basic science and clinical research, the pathways behind the immune-mediated destruction of pla ...
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Journal articleJ Biol Chem · September 24, 1999
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In hematopoietic cells, the signals initiated by activation of the phosphoinositide 3-kinase (PI3K) family have been implicated in cell proliferation and survival, membrane and cytoskeletal reorganization, chemotaxis, and the neutrophil respiratory burst. ...
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Journal articleMol Cell Biol · August 1998
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Reorganization of the actin cytoskeleton is an early cellular response to a variety of extracellular signals. Dissection of pathways leading to actin rearrangement has focused largely on those initiated by growth factor receptors or integrins, although sti ...
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