Search Results - "Höchsmann, Britta"
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Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria
Published in The New England journal of medicine (18-03-2021)“…Anemia associated with PNH is caused by hemolysis. The C5 inhibitor eculizumab blocks intravascular hemolysis, but anemia often persists owing to extravascular…”
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Incomplete inhibition by eculizumab: mechanistic evidence for residual C5 activity during strong complement activation
Published in Blood (23-02-2017)“…Eculizumab inhibits the terminal, lytic pathway of complement by blocking the activation of the complement protein C5 and shows remarkable clinical benefits in…”
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Eculizumab in Pregnant Patients with Paroxysmal Nocturnal Hemoglobinuria
Published in The New England journal of medicine (10-09-2015)“…Pregnancy has been discouraged in patients with paroxysmal nocturnal hemoglobinuria, a life-threatening hemolytic anemia, because of the heightened risk…”
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4
Complement and inflammasome overactivation mediates paroxysmal nocturnal hemoglobinuria with autoinflammation
Published in The Journal of clinical investigation (01-12-2019)“…Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a clonal population of blood cells deficient in glycosylphosphatidylinositol-anchored…”
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Haploidentical hematopoietic stem cell transplantation in aplastic anemia: a systematic review and meta-analysis of clinical outcome on behalf of the severe aplastic anemia working party of the European group for blood and marrow transplantation (SAAWP of EBMT)
Published in Bone marrow transplantation (Basingstoke) (01-10-2020)“…Aplastic anemia (AA) is a serious hematological disorder, which is solely cured by hematopoietic stem cell transplantation (HSCT). Haploidentical HSCT is an…”
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Constitutional PIGA mutations cause a novel subtype of hemochromatosis in patients with neurologic dysfunction
Published in Blood (03-03-2022)“…Muckenthaler et al describe a novel form of hemochromatosis caused by a constitutional PIGA mutation in 3 children with associated neurologic dysfunction…”
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Targeted Therapy with Eculizumab for Inherited CD59 Deficiency
Published in The New England journal of medicine (02-01-2014)“…Patients with CD59 deficiency have progressive neurologic dysfunction and episodes of hemolytic anemia. Eculizumab, a complement inhibitor, was administered to…”
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Deregulation of Factor H by Factor H-Related Protein 1 Depends on Sialylation of Host Surfaces
Published in Frontiers in immunology (25-02-2021)“…To discriminate between self and non-self surfaces and facilitate immune surveillance, the complement system relies on the interplay between surface-directed…”
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Long-term outcome of a randomized controlled study in patients with newly diagnosed severe aplastic anemia treated with antithymocyte globulin and cyclosporine, with or without granulocyte colony-stimulating factor: a Severe Aplastic Anemia Working Party Trial from the European Group of Blood and Marrow Transplantation
Published in Haematologica (Roma) (01-05-2020)“…This follow-up study of a randomized, prospective trial included 192 patients with newly diagnosed severe aplastic anemia receiving antithymoglobulin and…”
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Pegcetacoplan versus eculizumab in patients with paroxysmal nocturnal haemoglobinuria (PEGASUS): 48-week follow-up of a randomised, open-label, phase 3, active-comparator, controlled trial
Published in The Lancet. Haematology (01-09-2022)“…BACKGROUNDIn the PEGASUS trial, the complement C3 inhibitor, pegcetacoplan, showed superiority to eculizumab in improving haematological outcomes in adult…”
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Pegcetacoplan versus Eculizumab in Paroxysmal Nocturnal Hemoglobinuria
Published in The New England journal of medicine (18-03-2021)Get full text
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Response to Comment on "Self versus Nonself Discrimination by the Soluble Complement Regulators Factor H and FHL-1"
Published in The Journal of immunology (1950) (15-10-2019)Get full text
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Successful use of eculizumab for treatment of an acute hemolytic reaction after ABO-incompatible red blood cell transfusion
Published in Transfusion (Philadelphia, Pa.) (01-03-2015)“…Background Transfusion of ABO major–incompatible red blood cells (RBCs) can activate the complement system and can cause severe and even lethal acute hemolytic…”
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Comparative Analysis of Novel Complement-Targeted Inhibitors, MiniFH, and the Natural Regulators Factor H and Factor H-like Protein 1 Reveal Functional Determinants of Complement Regulation
Published in The Journal of immunology (1950) (15-01-2016)“…The serum proteins factor H (FH), consisting of 20 complement control protein modules (CCPs), and its splice product FH-like protein 1 (FHL-1; consisting of…”
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A case of paroxysmal nocturnal hemoglobinuria caused by a germline mutation and a somatic mutation in PIGT
Published in Blood (15-08-2013)“…To ascertain the genetic basis of a paroxysmal nocturnal hemoglobinuria (PNH) case without somatic mutations in PIGA, we performed deep next-generation…”
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Different Levels of Incomplete Terminal Pathway Inhibition by Eculizumab and the Clinical Response of PNH Patients
Published in Frontiers in immunology (18-07-2019)“…Eculizumab blocks the lytic complement pathway by inhibiting C5 and has become the standard of care for certain complement-mediated diseases. Previously, we…”
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Congenital CD59 Deficiency
Published in Hematology/oncology clinics of North America (01-06-2015)“…The severe clinical symptoms of inherited CD59 deficiency confirm the importance of CD59 as essential complement regulatory protein for protection of cells…”
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Bacterial contamination of platelet concentrates: results of a prospective multicenter study comparing pooled whole blood-derived platelets and apheresis platelets
Published in Transfusion (Philadelphia, Pa.) (01-04-2007)“…BACKGROUND: The GERMS Group initiated a prospective multicenter study to assess prevalence and nature of bacterial contamination of pooled buffy‐coat platelet…”
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A new blood group antigen is defined by anti-CD59, detected in a CD59-deficient patient
Published in Transfusion (Philadelphia, Pa.) (01-07-2014)“…Background CD59 is a cell surface glycoprotein of approximately 20 kDa limiting the lytic activity of the terminal complement complex C5b‐9. Although CD59 is…”
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Upfront Alternative Donor Transplant versus Immunosuppressive Therapy in Patients with Severe Aplastic Anemia Who Lack a Fully HLA-Matched Related Donor: Systematic Review and Meta-Analysis of Retrospective Studies, on Behalf of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation
Published in Transplantation and cellular therapy (01-02-2022)“…•Hematopoietic stem cell transplantation from a matched sibling donor (MSD) is the standard of care treatment for children and young adults with severe…”
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