PNH - 2
Defects cont’d:
This is the only acquired hemolytic anemia caused by
an abnormality of cell membrane
Defective gene:
Phosphatidylinositol, class A (PIGA)
On chromosome:
X chromosome
Specific hematopoitic cell affected:
Cells with increased susceptibility to lysis by
complement
Pluirpotential stem cell
Result of defects: