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  1. A Prospective Multicenter Study of Paroxysmal Nocturnal Hemoglobinuria Cells in Patients with Bone Marrow Failure.

    ... complement activation leading to intravascular hemolysis and an inflammatory prothrombotic state. The EXPLORE study aimed ... (BMF) syndromes and the effect of PNH clone size on hemolysis. Methods: Patients, selected from medical office chart reviews, had ...

    Research Article last updated 07/20/2018 - 5:15pm.

  2. A prospective multicenter study of paroxysmal nocturnal hemoglobinuria cells in patients with bone marrow failure.

    ... complement activation leading to intravascular hemolysis and an inflammatory prothrombotic state. The EXPLORE study aimed ... (BMF) syndromes and the effect of PNH clone size on hemolysis. METHODS: Patients, selected from medical office chart reviews, ...

    Research Article last updated 07/20/2018 - 5:15pm.

  3. Clinical roundtable monograph: Paroxysmal nocturnal hemoglobinuria: a case-based discussion.

    ... acquired disorder characterized by chronic intravascular hemolysis as the primary clinical manifestation and morbidities that ... platelets, resulting in complement-mediated intravascular hemolysis and other complications. Lysis of red blood cells is the most obvious ...

    Research Article last updated 07/20/2018 - 5:15pm.

  4. Paroxysmal nocturnal hemoglobinuria in pediatric patients.

    ... PNH include: bone marrow failure , intravascular hemolysis , and thrombosis . To further characterize the clinical ... in all patients with AA, MDS, unexplained Coombs-negative hemolysis, or thrombosis. While HSCT remains the only curative option the high ...

    Research Article last updated 07/20/2018 - 5:14pm.

  5. Complement C3dg-mediated erythrophagocytosis: implications for paroxysmal nocturnal hemoglobinuria

    ... that prevents complement-mediated intravascular hemolysis . However, a considerable fraction of PNH patients show ... we provide experimental evidence for such extravascular hemolysis and demonstrate that PNH erythrocytes from anti-C5-treated patients ...

    Research Article last updated 07/20/2018 - 5:15pm.

  6. Assessing complement blockade in patients with paroxysmal nocturnal hemoglobinuria receiving eculizumab

    ... (PNH) is characterized by intravascular hemolysis , effectively controlled with eculizumab , a humanized ... during treatment. From 2010 to 2012, clinical data, hemolysis biomarkers, complement assessment and free eculizumab circulating ...

    Research Article last updated 07/20/2018 - 5:15pm.

  7. Complement Blockade with a C1 Esterase Inhibitor in Paroxysmal Nocturnal Hemoglobinuria

    ... These patients experience both intra- and extravascular hemolysis in the context of underlying complement activation. Currently eculizumab effectively blocks the intravascular hemolysis PNH. There remains an unmet clinical need for a complement inhibitor ...

    Research Article last updated 07/20/2018 - 5:15pm.

  8. Long term treatment with eculizumab in paroxysmal nocturnal hemoglobinuria: sustained efficacy and improved survival.

    ... mortality and morbidity due to intense intravascular hemolysis . Eculizumab , a monoclonal antibody against the complement protein C5, stops the intravascular hemolysis in PNH. We evaluated 79 consecutive patients treated with eculizumab ...

    Research Article last updated 07/20/2018 - 5:14pm.

  9. How I treat paroxysmal nocturnal hemoglobinuria.

    ... disease result from complement-mediated intravascular hemolysis . Allogeneic bone marrow transplantation is the only curative ... complement activation, is highly effective in reducing hemolysis, improving quality of life, and reducing the risk for thrombosis in ...

    Research Article last updated 07/20/2018 - 5:14pm.

  10. Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review

    ... action of complement. Patients experience intravascular hemolysis , smooth muscle dystonia, renal failure, arterial and pulmonary ... of eculizumab , which brings benefits in terms of hemolysis, quality of life, renal function, thrombotic risk, and life ...

    Research Article last updated 07/20/2018 - 5:15pm.