Complement C3dg-mediated erythrophagocytosis: implications for paroxysmal nocturnal hemoglobinuria.

ABSTRACT: The clinical management of paroxysmal nocturnal hemoglobinuria (PNH), a rare but life-threatening hematologic disease, has fundamentally improved with the introduction of a therapeutic that prevents complement-mediated intravascular hemolysis. However, a considerable fraction of PNH patients show insufficient treatment response and remain transfusion dependent. Because the current treatment only prevents C5-induced lysis but not upstream C3 activation, it has been speculated that ongoing opsonization with C3 fragments leads to recognition and phagocytosis of PNH erythrocytes by immune cells. Here, for the first time, we provide experimental evidence for such extravascular hemolysis and demonstrate that PNH erythrocytes from anti-C5-treated patients are phagocytosed by activated monocytes i

SUBMITTER: Lin Z 

PROVIDER: S-EPMC4536542 | biostudies-literature | 2015 Aug

REPOSITORIES: biostudies-literature