Transfusion of patients who have paroxysmal nocturnal hemoglobinuria (PNH)

A blood banker asked whether or not it was necessary to transfuse patients who have paroxysmal nocturnal hemoglobinuria (PNH) with saline-washed red blood cells. She asked this question because many blood bankers advocate transfusing PNH patients with saline-washed red blood cells. She indicates that her center currently supports two patients with PNH, and that her center is wondering if it would be safe to transfuse PNH patients with standard RBC units, rather than with saline-washed red cells. According to the editor of the future 14th edition of the AABB Technical Manual, PNH red cells have an inability to down regulate small amounts of complement activation, and that in the 1940s, Sir John Dacie transfused a PNH patient with a unit of group O whole blood (which contained recipient ABO-incompatible plasma) and the transfusion was complicated by an exacerbation of the patient's hemolysis and subsequently became the basis for the recommendation to wash red cells for transfusion. A similar episode of increased hemolysis in a PNH patient following transfusion of a unit of group O whole blood (which also contained recipient ABO-incompatible plasma) occurred at the Mayo Clinic in 1950 (although reported decades later). The thought was that the addition of fresh complement would lead to an exacerbation of a patient's hemolysis. The current thinking is that in these two transfusions the infusion of ABO-incompatible plasma led to activation of complement. In a normal patient, the amount of complement activation related to the infusion of one unit of plasma would be down regulated and transfusion-induced clinical hemolysis should not be observed. However, in a PNH patient, such down regulation is not possible and they have hemolysis. Interestingly, according to the Technical Manual editor, PNH patients frequently suffer GI bleeds (thought to be secondary to platelet abnormalities and a propensity for thrombosis), and are transfused with group-specific plasma without increases in hemolysis. In a review from the Mayo Clinic of thirty-eight years of experience (1950 through 1987) with 23 transfused PNH patients (transfused with 556 blood components), only one documented episode of post-transfusion hemolysis related to the underlying diagnosis of PNH was found (as described above, associated with the transfusion of a unit of group O whole blood to a group AB-positive individual). This study concluded that the use of saline-washed RBCs is unnecessary and that patients with PNH may be transfused with group-specific blood and blood products. The e-network is referred to the article by Brecher ME, Taswell HF. Paroxysmal nocturnal hemoglobinuria and the transfusion of washed red cells. A myth revisited. Transfusion. 1989 Oct;29(8):681-5 and the accompanying editorial by Rosse W. Transfusion in paroxysmal nocturnal hemoglobinuria. To wash or not to wash? Transfusion. 1989 Oct;29(8):663-4.

The following replies were submitted in response to the above query and discussion:

1. A blood banker from Philadelphia commented that based on a review of the literature and the Brecher / Taswell article cited above it seems that one should avoid giving ABO-incompatible platelet products to PNH patients.
   
   
2. In harmony with reply #1 above, a blood banker from North Carolina wrote that group-specific blood components (RBCs, platelets, plasma, etc.) are the desired products for transfusion in this disease.
   
   
3. A blood banker from England reports that the National Blood Service in England recommends (and provides) standard red cells in OAS for patients with PNH. According to follow-up, no untoward effects have been reported. (If anyone knows what OAS is, please contact the Editor.
   
   ADDENDUM: According to a blood banker from England, OAS stands for Optimum Additive Solution. In the UK, SAG-M (saline, adenine and glucose) is used and is comparable to AS-1 (Adsol).

ADDENDA Jan. 24, 2002

4. An Australian blood banker wrote that the article by Brecher & Taswell (see above) challenged to the need to wash RBCs for PNH patients. In addition, based on (1) the review in J Dacie. PNH. In: The Haemolytic Anaemias Vol 5: Drug- and chemical-induced haemolytic anaemias; Paroxysmal nocturnal haemoglubinuria; Haemolytic disease of the newborn. 3rd ed 1999; chapter 55: 285-330, and (2) opinion from an expert, by P Hillmen. PNH. In: Recent advances in Haematology. 1996; chapter 7: 137-157, his center does not automatically provide washed RBCs to patients with PNH, but reserves washed red cells for patients with problems from "group-specific leukodepleted" blood products. In their last PNH patient who had episodic haemolysis, Buddi-Chiari syndrome, and a stem cell allograft, he did not have any haemolysis from leukodepleted RBCs (despite alleged haemolysis from unwashed RBC given in another hospital). He had a minor haemolysis temporally related to FFP given for the correction of coagulopathy. A logistic argument for not giving routine washed RBCs to PNH patients undergoing transplantation is their need of platelet support.

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