Freeze-cleaving of red cell membranes in paroxysmal nocturnal hemoglobinuria.

Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...

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...

Electron microscope study of PNH red cells and AET-treated normal red cells (PNH-like cells).

The morphology of red cells damaged by 2-aminoethylisothiouronium bromide (AET) has been compared to that of PNH cells by transmission and scanning electron microscopy. The main features of the effect of AET as demonstrated by the scanning electron microscope were spherical and deformed cells, with surface craters and relatively large pits. The transmission electron microscope revealed loss of ...

The life-span of complement-sensitive and -insensitive red cells in paroxysmal nocturnal hemoglobinuria.

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.