G-CSF and cyclosporin induce an increase of normal cells in hypoplastic paroxysmal nocturnal hemoglobinuria
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Keywords:
Paroxysmal nocturnal hemoglobinuria
Hemoglobinuria
Aplastic anemia
CD59
Hematology
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disease with complex pathophysiology, characterized by a global deficiency in glycosyl-phosphatidylinositol-anchored proteins in the affected cells. The name of the condition emphasizes the occurrence of complement-induced hemolysis due to lack of essential complement regulatory proteins in erythrocytes, particularly CD59. Over time, it became apparent that episodic exacerbations of chronic hemolytic anemia are not necessarily nocturnal, nor do they represent the main presenting symptom in all cases. Paroxysmal nocturnal hemoglobinuria can also manifest with thrombotic events and bone marrow failure, and distinguishing among different types of PNH is important for treatment and prognostic purposes.
Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
Eculizumab
Bone marrow failure
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Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
Eculizumab
Hematopoietic stem cell
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Objective:To study the significance of Decay Accelerating Factor(DAF=CD55)?Membrane Inhibitor of reactive Lysis (MIRL=CD59) in the diagnostic of paroxysmal nocturnal hemoglobinuria (PNH). Methods: Flow cytometric analysis of CD55?CD59 was carried to detect the deficiency of membrane protein to the cell surface. Results: The level of CD55(45.94±6.06)%,CD59(46.40±12.36)% of PNH group was significantly lower than that of in AA-PNH CD55(71.00±0.43)%,CD59(84.62±2.08)%,in AA CD55(99.19±0.86)%,CD59(94.42±2.92)% and control group CD55(99.23±0.95)%,CD59(98.56±1.25)%.Conclusion: CD55 and CD59 is a useful target to diagnose and characterize PNH.
Paroxysmal nocturnal hemoglobinuria
CD59
Decay-accelerating factor
Hemoglobinuria
Eculizumab
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Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
CD5
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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by an intravascular hemolytic anemia. Abnormal blood cells lack a series of glycosylphosphatidylinositol (GPI)-anchored proteins. The lack of GPI-anchored complement regulatory proteins, such as decay-accelerating factor (DAF) and CD59, results in complement-mediated hemolysis and hemoglobinuria. In the affected hematopoietic cells from patients with PNH, the first step in biosynthesis of the GPI anchor is defective. At least four genes are involved in this reaction step, and one of them, an X-linked gene termed PIG-A, is mutated in affected cells. The PIG-A gene is mutated in all patients with PNH reported to date. Here, we review recent advances in the understanding of the molecular pathogenesis of PNH. Am. J. Hematol. 62:175–182, 1999. © 1999 Wiley-Liss, Inc.
Paroxysmal nocturnal hemoglobinuria
Hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria is a rare clonal disease of hematopoietic stem cells. These hematopoietic cells present a deficit of surface membrane anchorage proteins such as DAF (Complement Degradation Accelerator Factor, CD55) and MIRL (Membrane Reactive Lysis Inhibitor, CD59). This total or partial absence produces a greater susceptibility of erythrocytes to complement-mediated hemolysis. Eculizumab is a monoclonal antibody indicated in the paroxysmal nocturnal hemoglobinuria, capable of blocking the complement protein C5 avoiding its activation and, therefore, hemolysis. We present the case of a woman diagnosed with paroxysmal nocturnal hemoglobinuria resistant to the use of systemic corticosteroids with a poor quality of life, an important amount of symptoms and hospitalizations in several occasions. After eight years in treatment with eculizumab the patient presents a good drug tolerance, a better quality of life and a decrease in the value of lactate dehydrogenase, stabilization of the value of hemoglobin and a reduction on the number of units of packed red cells transfused
Paroxysmal nocturnal hemoglobinuria
Eculizumab
Hemoglobinuria
CD59
Decay-accelerating factor
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Background : Paroximal nocturnal hemoglobinuria (PNH) is a disorder of the pluripotent stem cells resulting in a deficient expression of membrane-bound GPI-anchored proteins in different cell types. We evaluated REDQUANT and CELLQUANT kits (Biocytex, Marseille, France) for PNH test. Methods : Seventy patients with peripheral blood cytopenia and 16 healthy controls were studied. RBCs and granulocytes were tested for CD55 and CD59 expression using the REDQUANT and CELLQUANT kits and an Epics XL flow cytometer. According to the manufacturer’s instruction, results were interpreted abnormal when more than 3% of cells were deficient in the expression of CD55 or CD59, and a test was considered positive for PNH if three of the four markers tested were abnormal. Results : The percentage of CD55/CD59 deficient RBCs and granulocytes was 0.3/3.1 and 3.5/ 10.0, respectively, in the patient group, and 0.1/1.0 and 0.3/9.7, respectively, in the control group. PNH was diagnosed in three patients who had a deficiency in the expression of three or four antigens; two other patients showed a deficiency in two antigens. There were many who had CD59 deficiency only: on granulocytes in 30 patients and 11 controls, and on RBCs in 6 patients and 2 controls. One patient had CD55 deficient granulocytes. Conclusion : The REDQUANT/CELLQUANT kit is a standardized method and does not require normal samples as the control, but one should be cautious in interpreting the results showing CD59 expression on granulocytes.
Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
Aplastic anemia
Cytopenia
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Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disease with complex pathophysiology, characterized by a global deficiency in glycosyl-phosphatidylinositol-anchored proteins in the affected cells. The name of the condition emphasizes the occurrence of complement-induced hemolysis due to lack of essential complement regulatory proteins in erythrocytes, particularly CD59. Over time, it became apparent that episodic exacerbations of chronic hemolytic anemia are not necessarily nocturnal, nor do they represent the main presenting symptom in all cases. Paroxysmal nocturnal hemoglobinuria can also manifest with thrombotic events and bone marrow failure, and distinguishing among different types of PNH is important for treatment and prognostic purposes.
Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
Eculizumab
Bone marrow failure
Cite
Citations (1)