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A Simplified Assay for the Specific Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Detection of DAF(CD55)‐ and HRF20(CD59)‐ Erythrocytes in Microtyping Cards

Bo Nilsson, Ulla Hagström, Åsa Englund, Jan Säfwenberg

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Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that is caused by a monoclonal stem cell defect. The affected cells lack the carbohydrate linkage between phosphatidylinositol and a group of membrane proteins of which three protect the cell against complement lysis. The absence of these three proteins, DAF(CD55), C8BP and HRF20(CD59), makes cells from the erythropoiesis, thrombopoiesis and myelopoiesis extensively sensitive to complement attack and affected patients suffer from intravascular hemolysis, thrombosis and increased susceptibility to infections. In this study we describe a swift and specific assay for the detection of CD55- and CD59- erythrocytes, which is suitable for screening of possible PNH patients.

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What this paper is about

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that is caused by a monoclonal stem cell defect. The affected cells lack the carbohydrate linkage between phosphatidylinositol and a group of membrane proteins of which three protect the cell against complement lysis. The absence of these three proteins, DAF(CD55), C8BP and HRF20(CD59), makes cells from the erythropoiesis, thrombopoiesis and myelopoiesis extensively sensitive to complement attack and affected patients suffer from intravascular hemolysis, thrombosis and increased susceptibility to infections. In this study we describe a swift and specific assay for the detection of CD55- and CD59- erythrocytes, which is suitable for screening of possible PNH patients.

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Available abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease that is caused by a monoclonal stem cell defect. The affected cells lack the carbohydrate linkage between phosphatidylinositol and a group of membrane proteins of which three protect the cell against complement lysis. The absence of these three proteins, DAF(CD55), C8BP and HRF20(CD59), makes cells from the erythropoiesis, thrombopoiesis and myelopoiesis extensively sensitive to complement attack and affected patients suffer from intravascular hemolysis, thrombosis and increased susceptibility to infections. In this study we describe a swift and specific assay for the detection of CD55- and CD59- erythrocytes, which is suitable for screening of possible PNH patients.

Key concepts: Paroxysmal nocturnal hemoglobinuria, CD59, Eculizumab, Hemoglobinuria, Hemolysis, Complement membrane attack complex, Immunology, Decay-accelerating factor

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A Simplified Assay for the Specific Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Detection of DAF(CD55)‐ and HRF20(CD59)‐ Erythrocytes in Microtyping Cards — Research Paper | ScholarLens