It was suggested that chronic arterial thrombosis secondary to autoimmunity may be the main cause of MMS formation.[2],[3] In PNH patient, deficiency of two important complement regulatory proteins on cell surfaces,
CD55 and CD59, makes blood cells more sensitive and vulnerable to the action of complement response.
Cromer is located on decay accelerating factor (DAF,
CD55) which inhibits assemblage of C3 and C5 converting enzymes of the classical and alternative pathways.
Clusters 1 and 2 are characterized by significant overexpression of genes responsible for extracellular matrix remodeling and cell recruitment (for example, matrix metalloproteinase 3 (Mmp3; p < [10.sup.-7]), collagen 1a2 (Col1a2; p < [10.sup.-4]), and stromal cell factor 1 (Cxcl12/Sdf1; p < [10.sup.-3]) (Figure 2(a)) as well T cell and complement activation (dipeptyl-peptide4 (Dpp4/Cd26; p < [10.sup.-7]) and
Cd55; p < [10.sup.-6]) (Figure 2(b)).
Paroxysmal nocturnal hemoglobinuria (PNH) is a complex hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan (PIG-A) gene resulting in a deficiency of the glycosyl phosphatidylinositol (GPI) anchored proteins, particularly
CD55 and CD59 on the cell membrane [1].
ID MARKET $/GAL ID A CONTRACTOR 15.0 Ac15 A CONTRACTOR 17.5 Ac17 A DIY 25.0 Ad25 A DIY 42.0 Ad42 B CONTRACTOR 22.5 Bc22 B CONTRACTOR 42.0 Bc42 B DIY 42.1 Bd42 B DIY 44.8 Bd44 C CONTRACTOR 12.6 Cc12 C CONTRACTOR 20.6 Cc20 C DIY 21.6 Cd21 C DIY 55.4
Cd55 D CONTRACTOR 40.4 Dc40 D CONTRACTOR 41.6 Dc41 D DIY 40.6 Dd40 D DIY 69.8 Dd69 TABLE 2--Painting Experience Evaluation STRONGLY DISAGREE DISAGREE STEADY 1.
In view of initial reports showing hemolysis and presence of cerebral sinus thrombus, a rare differential diagnosis of PNH was kept and flow cytometry was performed on granulocytes and RBCs, revealing that 95.6% granulocytes and 94% of RBCs were negative for
CD55.