The use of monoclonal antibodies and flow cytometry in the diagnosis of paroxysmal nocturnal hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria
CD59
Hemoglobinuria
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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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)
To investigate the relationship between CD59 and traditional hemolysis tests for paroxysmal nocturnal hemoglobinuria.To make different concentrations of CD59-blocked erythrocytes by mixing with normal erythrocytes, and investigate the relationship between quantitative abnormalities of CD59 and hemolysis tests.The hemolysis rate was positively related to the percentage of CD59-blocked erythrocytes. The greatest hemolysis of CD59-blocked erythrocytes was found in the CoF-initiated hemolysis test when the CD59-defects less than 16%, and the greater in Ham's test. When the CD59 defects were more than 16%, CD59-blocked erythrocytes hemolyzed to a greater extent in Ham's test than in the others.Quantitative differences of CD59 underlies the variability in hemolysis tests, and the CoF-initiated hemolysis test is the most sensitive in detection of small CD59-defects.
Paroxysmal nocturnal hemoglobinuria
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Hemoglobinuria
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