RED CELLS, IRON, AND ERYTHROPOIESIS Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab
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چکیده
1Hematology, Department of Biochemistry and Medical Biotechnologies, Federico II University, Naples; 2Laboratory of Genetics and Gene Transfer, Core Research Laboratory–Istituto Toscano Tumori (CRL-ITT), Florence; 3Hematology, University of Florence, Florence; 4Department of Cellular Biotechnologies and Hematology, La Sapienza University, Rome; 5Department of Hematology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan; 6Department of Cell Therapy and Hematology, Hemophilia and Thrombosis Center, San Bortolo Hospital, Vicenza; 7Immunohematology and Transfusion Medicine, Federico II University, Naples; 8Department of Nuclear Medicine, IRCCS Hospital Casa Sollievo Della Sofferenza, San Giovanni Rotondo; and 9Istituto Toscano Tumori (ITT), Florence, Italy
منابع مشابه
RED CELLS, IRON, AND ERYTHROPOIESIS A novel approach to preventing the hemolysis of paroxysmal nocturnal hemoglobinuria: both complement-mediated cytolysis and C3 deposition are blocked by a monoclonal antibody specific for the alternative pathway of complement
The clinical hallmark of paroxysmal nocturnal hemoglobinuria (PNH) is chronic intravascular hemolysis that is a consequence of unregulated activation of the alternative pathway of complement (APC). Intravascular hemolysis can be inhibited in patients by treatment with eculizumab, a monoclonal antibody that binds complement C5 thereby preventing formation of the cytolytic membrane attack complex...
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Ferrokinetics data on patients with blood disorders of diverse etiology were presented and the clinical implication of this test was discussed. Ferrokinetics data on normal adults as well as on the aged were also presented. Ferrokinetic patterns of ineffective erythropoiesis were obtained on patients with pernicious anemia, erythroleukemia, myelofibrosis, paroxysmal nocturnal hemoglobinuria and...
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Glucocorticoids are used extensively to treat autoimmune hemolytic anemias. Some beneficial effects of glucocorticoid pulse therapy have also been reported in sickle cell disease and paroxysmal nocturnal hemoglobinuria. Based on established concepts of hemoglobin (Hb) toxicity and physiologic Hb scavenger systems, we evaluated whether glucocorticoids could support an adaptive response to extrac...
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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia that results from the expansion of hematopoietic stem cells that are deficient for glycosylphosphatidylinositol (GPI), a glycolipid moiety that anchors .100 different proteins to the cell surface. PNH patients were reported to be deficient for an initial step in the GPI anchor synthesis that is catalyzed by the GPI-GlcNAc...
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Eryptosis, the suicidal death of erythrocytes, is characterized by erythrocyte shrinkage, blebbing, and phospholipid scrambling of the cell membrane. Eryptosis is triggered by increased cytosolic Ca(2+) activity, which may result from Ca(2+) entry through PGE(2)-activated Ca(2+)-permeable cation channels. The Ca(2+) sensitivity of the scrambling machinery is enhanced by ceramide, which is forme...
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تاریخ انتشار 2009