Immunophenotypic analysis of PNH cells.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem-cell disorder in which a gene mutation results in cellular inability to synthesize the glycosylphosphatidylinositol (GPI) anchors needed to attach various proteins to the cell membrane. Traditional clinical laboratory PNH screening and diagnosis are based upon demonstration of increased erythrocyte susceptibility to lysis by activated complement. These methods are neither very specific nor very sensitive, and have largely been superseded by the rapid, sensitive, and specific flow cytometric analysis of GPI-linked antigen expression. The protocols in this unit cover immunophenotypic analysis of red blood cells, peripheral blood granulocytes, and peripheral blood monocytes, as well as a four-color modification for simultaneous analysis of peripheral blood granulocytes and monocytes.
Richards
Stephen J
SJ
Haematological Malignancy Diagnostic Service, Leeds, United Kingdom.
Hillmen
Peter
P
D018957:CD59 Antigens / Q000097:blood; D004912:Erythrocytes / Q000473:pathology; D017261:Glycosylphosphatidylinositols / Q000378:metabolism; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000473:pathology*; D006801:Humans; D016130:Immunophenotyping / Q000379:methods*; D009000:Monocytes / Q000473:pathology; D009504:Neutrophils / Q000473:pathology
D018957:CD59 Antigens; D017261:Glycosylphosphatidylinositols