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Figure 1.

Figure 1. From: Paroxysmal nocturnal hemoglobinuria and eculizumab*.

The complement cascade regulation and erythrocytes. (A) Normal erythrocytes are protected from complement activation and subsequent hemolysis by CD55 and CD59. These two proteins, being GPI-linked, are missing from the surface of PNH red cells as a result of a somatic mutation of the X-linked PIG-A gene, that encodes a protein required for an early step of the GPI molecule biosynthesis.6 (B) In the steady state, PNH erythrocytes suffer from spontaneous (tick-over) complement activation, with consequent intravascular hemolysis through formation of the MAC; exacerbated hemolysis will result from activation of extra complement through the classical pathway. (C) On eculizumab, PNH erythrocytes are protected from hemolysis from the inhibition of C5 cleavage; however, upstream complement activation may lead to C3 opsonization and possible extravascular hemolysis. Here “C3 convertase” represents either the C3bBb complex (alternative pathway) or the C4b2a complex (classical and lectin pathways).

Lucio Luzzatto, et al. Haematologica. 2010 April;95(4):523-526.

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