Effect of heparin on complement activation and lysis of paroxysmal nocturnal hemoglobinuria (PNH) red cells.

Immune lysis of normal human and paroxysmal nocturnal hemoglobinuria (PNH) red blood cells. I. The sensitivity of PNH red cells to lysis by complement and specific antibody.

The pathophysiology of disease in patients with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia caused by the expansion of a hematopoietic progenitor cell that has acquired a mutation in the X-linked PIGA gene. PNH occurs on the background of bone marrow failure. Bone marrow failure and the presence of the abnormal cells account for the clinical phenotype of patients with PNH including hemolysis, cytopenia, and thro...

Inhibitors of complement derived from the erythrocyte membrane in paroxysmal nocturnal hemoglobinuria.

Extracts of the membranes of normal red cells and red cells from all subpopulations of paroxysmal nocturnal (PNH) red cells inhibited antibody-mediated complement activation. These extracts were shown to accelerate decay of the complement complex. C42, and the relative amount of inhibitory activity was similar in normal and PNH membranes. Inhibitors derived from normal red cells markedly decrea...

Leukocyte antigen-antibody reaction and lysis of paroxysmal nocturnal hemoglobinuria erythrocytes.

T HE TRANSFUSION OF COMPATIBLE WHOLE BLOOD to patients suffering from paroxysmal nocturnal hemoglobinuria ( PNH ) is known to sometimes cause a hemolytic reaction. It has been suggested that leukocyte isoantibodies present in the patient’s serum through interaction with the donor’s leukocyte antigens are responsible for lysis of PNH red cells.’ As yet, it has not been clarified why this interac...

Differences in the terminal steps of complement lysis of normal and paroxysmal nocturnal hemoglobinuria red cells.

The number of microscopically visible lesions produced on the membrane for a given degree of lysis on normal cells as on PNH cells. Since complement lesions were not formed until C8 or C9 was incorporated into the complement sequence, the results suggest that increased lysis of red cells in PNH is due at least in part to more efficient penetration of the PNH membrane by the terminal lytic seque...