Perioperative management of patients with paroxysmal nocturnal hemoglobinuria

During the past two decades, we have had the opportunity to study a large number of patients with the rare hemolytic anemia, paroxysmal nocturnal hemoglobinuria. Sixteen surgical procedures have been performed upon ten patients with paroxysmal nocturnal hemoglobinuria. From this experience, we have developed certain recommendations for preoperative evaluation and preparation, intraoperative management and post-operative care. Any proposed surgical procedure for a patient with paroxysmal nocturnal hemoglobinuria should be closely managed by a team consisting of a surgeon, hematologist and anesthesiologist. In the preoperative evaluation, particular attention should be paid to the hematologic, renal and hepatic status. Transfusion requirements, whether precipitated by hemolytic episodes or surgical blood loss, should be met with either washed or perviously frozen erythrocytes, with consideration of the use of dextran for volume replacement. Dextran is the treatment of choice for thrombotic epidsodes. Close attention should be given to avoiding hypoxia and dehydration. Anesthetic agents which may activate complement or may be hepatotoxic are to be avoided.