Article
New insights into paroxysmal nocturnal hemoglobinuria.
Hematology. American Society of Hematology. Education Program - 1 Jan 2006
Brodsky Robert A
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon intravascular hemolytic anemia that results from the clonal expansion of hematopoietic stem cells harboring somatic mutations in an X-linked gene, termed PIG-A. PIG-A mutations block glycosylphosphatidylinositol (GPI) anchor biosynthesis, resulting in a deficiency or absence of all GPI-anchored proteins on the cell surface. CD55 and CD59 are GPI-anchored...
Topics
- Antibodies, Monoclonal
- Hematopoietic Stem Cells
- Hemoglobinuria, Paroxysmal
- Hemolysis
- Humans
- Membrane Proteins
- Mutation