A patient with paroxysmal nocturnal hemoglobinuria (PNH) faces a number of problems ini the perioperative period. Ogin reported recommendations for management of these patients. The author is from the University of Colorado.


Risk factors faced by patients with PNH:

(1) complement activiation that can trigger intravascular hemolysis

(2) deep vein thrombosis (DVT)


Things to avoid if possible in the perioperative period:

(1) hypoxemia

(2) acidosis

(3) dehydration

(4) drugs that activate complement (drugs formulated in Cremophor, magnesium compounds, acetazolamide, some radiocontrast materials)

(5) postoperative infection

(6) anaphylactioid reactions

(7) an acute phase reaction


Induction agents with a low risk of anaphylactoid reactions:

(1) benzodiazepines (midazolam, others)

(2) opioids (including fentanyl)

(3) thiopental


Prophylactic steps:

(1) steps to prevent deep vein thrombosis

(2) corticosteroids

(3) perioperative antibiotics

(4) careful wound care

(5) vigorous pulmonary toilet to help to avoid postoperative pneumonia

(6) blood transfusion if anemic or blood loss expected, preferably with washed red blood cells


Therapy if hemolysis occurs:

(1) blood transfusions

(2) corticosteroids


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