RBC transfusions in paroxysmal nocturnal hemoglobinuria

Abstract

Frozen-thawed, deglycerolized RBCs can be substituted for saline-washed RBCs for transfusion of patients with paroxysmal nocturnal hemoglobinuria (PNH). The effect of transfusion is to decrease bone marrow production of RBCS, which results in a decreased percentage of complement-sensitive erythrocytes. In the presence of small numbers of complement-sensitive erythrocytes, major vascular surgery can be performed without hemolytic problems. This study suggests that the percentage of complement-sensitive RBCs, as well as the complement sensitivity of these cells, influence the severity of hemolysis in persons with PNH.