PERIOPERATIVE MANAGEMENT OF PATIENTS WITH PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA

  • 1 January 1981
    • journal article
    • research article
    • Vol. 153  (4) , 515-520
Abstract
A large number of patients with the rare hemolytic anemia, paroxysmal nocturnal hemoglobinuria were studied. Sixteen surgical procedures were performed upon 10 patients with paroxysmal nocturnal hemoglobinuria. Recommendations for preoperative evaluation and preparation, intraoperative management and postoperative care are reported. 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 previously frozen erythrocytes, with consideration of the use of dextran for volume replacement. Dextran is the treatment of choice for thrombotic episodes. Close attention should be given to avoiding hypoxia and dehydration. Anesthetic agents which may activate complement or may be hepatotoxic are to be avoided.