Paroxysmal nocturnal hemoglobinuria

All patients with paroxysmal nocturnal hemoglobinuria (PNH) should be seen once a year, and more often if more ill. Patients requiring transfusion should be seen at least every 2 months. The size of the PNH clone should be evaluated by flow cytometry at least once a year. Patients receiving complement inhibitors should be seen every 3-6 months to monitor the effectiveness and toxicity of therapy. Breakthrough hemolysis, insufficient dose, and/or extravascular hemolysis should be strictly monitored and addressed by switching to alternative complement inhibition, increasing the dose, or by adding on proximal complement inhibition.