What is paroxysmal nocturnal hemoglobinuria (PNH)?

• Clonal deficiency in glycosyl phophatidyl inositol (GPI-linked protiens on RBCs, neutrophils, and platelets) GPI-linked proteins; which neutralize complements attached to RBC, neutrophils, and platelets - DAF, decay-accelerating factor (CD55) - MIRL, membrane-inhibitor reactive lysis (CD59) - In the absence of this protein, RBCs are susceptible to complementmediated intravascular hemolysis