Premium
A Simplified Assay for the Specific Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Detection of DAF(CD55) ‐ and HRF20(CD59) ‐ Erythrocytes in Microtyping Cards
Author(s) -
Nilsson Bo,
Hagström Ulla,
Englund Åsa,
Säfwenberg Jan
Publication year - 1993
Publication title -
vox sanguinis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.68
H-Index - 83
eISSN - 1423-0410
pISSN - 0042-9007
DOI - 10.1111/j.1423-0410.1993.tb02513.x
Subject(s) - paroxysmal nocturnal hemoglobinuria , cd59 , eculizumab , hemoglobinuria , hemolysis , complement membrane attack complex , immunology , decay accelerating factor , haptoglobin , erythroblast , red blood cell , myelopoiesis , monoclonal antibody , hemolytic anemia , ineffective erythropoiesis , biology , erythropoiesis , complement system , medicine , haematopoiesis , antibody , stem cell , anemia , microbiology and biotechnology
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 phophatidylinositol 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 HRF 20 (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.