Article
ICCS/ESCCA consensus guidelines to detect GPI-deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders part 1 - clinical utility.
Cytometry. Part B, Clinical cytometry - 1 Jan 2018
Dezern Amy E, Borowitz Michael J
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) arises as a consequence of the non-malignant clonal expansion of one or more hematopoietic stem cells with an acquired somatic mutation of the PIGA gene (Brodsky RA. Blood 113 (2009) 6522-6527). Progeny of affected stem cells are deficient in glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This deficiency is readily detected by flow cytometry. Though this seems...
Topics
- Adolescent
- Aged
- Clone Cells
- Disease Progression
- Female
- Flow Cytometry
- Glycosylphosphatidylinositols
- Hematopoietic Stem Cells
- Hemoglobinuria, Paroxysmal
- Humans
- Male