Paroxysmal nocturnal hematuria

# PNH results from somatic mutation in PIGA gene which leads to the shortage of GPI

# GPI anchors protective proteins (CD55 and CD59) to the surface of RBC
CD55 blocks C3bBb (C3 convertase from alternative pathway)
CD59 blocks the formation of MAC through binding to C8 and (C9)n

# Eculizumab binds specifically to C5 and blocks the action of C5 convertase
– RBC is still subject to C3b opsonization

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