نتایج جستجو برای: pnh
تعداد نتایج: 752 فیلتر نتایج به سال:
Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a large clonal population of blood cells deriving from hematopoietic stem cells (HSCs) deficient in glycosylphosphatidylinositol (GPI)-anchored surface molecules. A current model postulates that PNH arises through negative selection against normal HSCs exerted by autoreactive T cells, whereas PNH HSCs escape damage. We have investigat...
In paroxysmal nocturnal hemoglobinuria (PNH), acquired somatic mutations in the PIG-A gene give rise to clonal populations of red blood cells unable to express proteins linked to the membrane by a glycosylphosphatidylinositol anchor. These proteins include the complement inhibitors CD55 and CD59, and this explains the hypersensitivity to complement of red cells in PNH patients, manifested by in...
WENTY-FIVE YEARS AGO, William Dameshek, T the founder of this journal and one of the most creative minds in American hematology, raised a provocative question: what do aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and acute leukemia have in common?’ His question was prompted by three observations: (1) the frequency of development of PNH in his own patients with AA, (2) the ov...
In paroxysmal nocturnal hemoglobinuria (PNH) hemolytic anemia is due mainly to deficiency of the complement regulator CD59 on the surface of red blood cells (RBCs). Eculizumab, an antibody that targets complement fraction 5 (C5), has proven highly effective in abolishing complement-mediated intravascular hemolysis in PNH; however, the hematologic benefit varies considerably among patients. In t...
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematologic disorder with multiple and varied clinical manifestations. The biochemical defect in PNH resides in the incomplete enzymatic assembly of glycosylphosphatidylinositol (GPI) anchors used for surface protein attachment. In all patients tested thus far, the defect is at the level of N-acetylglucosamine attachment to phospha...
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...
Extracts of the membranes of normal red cells and red cells from all subpopulations of paroxysmal nocturnal (PNH) red cells inhibited antibody-mediated complement activation. These extracts were shown to accelerate decay of the complement complex. C42, and the relative amount of inhibitory activity was similar in normal and PNH membranes. Inhibitors derived from normal red cells markedly decrea...
C lones of paroxysmal nocturnal hemoglobinuria (PNH) cells – glycosylphosophoinositol (GPI)-anchored protein-deficient hematopoietic cells – can be detected by flow cytometry in patients with myelodysplastic sydromes (MDS). While the etiology of PNH is known to be a somatic mutation in the X-linked PIG-A gene, which abrogates GPI synthesis, the patho-physiology of PNH clonal expansion is not we...
N ABNORMALITY of the red cells of paroxysmal nocturnal hemoglobinuria ( PNH ) is responsible for their short life span in the circulation anu their lysis in vitro by certain factors present in normal plasma or serum. The hemolytic system in vitro is characteristically more active when the serum is made somewhat acid, about pH 6.8. The addition of thrombin to the serum is also capable of increas...
The association of paroxysmal nocturnal hemoglobinuria (PNH) and aplastic anemia (AA) raises the yet unresolved questions as to whether these two disorders are different forms of the same disease. We compared two groups of patients with respect to cytogenetic features, glycosylphosphatidylinositol (GPI)-linked protein expression, protein C/protein S/thrombomodulin/antithrombin III activity, and...
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