Should people with HAE be listed for potential bone marrow transplant donors? Should they give blood?

Jun 24, 2015

Dr. C: We would like to thank Janet Long for passing along this question from the HAEA. I believe that it was pointed out by one of the patients on the website that HAE may be too rare for people to think about with regard to these questions. Indeed checking the criteria for both bone marrow and blood donation requirements there is no mention of HAE as an exclusion. We have in a prior discussion however reviewed that a stem cell transplant was able to cure a patient with Evan’s Syndrome that was also suffering from HAE. The reverse could also be true—a bone marrow donation from a patient with HAE could cause the disease in the recipient. Bruce, any thoughts?

Dr. Z: We end up talking about this Evan’s Syndrome paper a lot, don’t we? I’d say that nobody really knows whether bone marrow from a HAE patient transplanted into a patient with a normal liver would reduce the level of C1 inhibitor.

Dr. C: I wonder if the C1 inhibitor produced by the bone marrow derived cells in the Evan’s Syndrome patient represented a compensatory response to the lack of C1 inhibitor from the liver?

Dr. Z: Yes, that’s what I’ve thought as well. On the other hand, we recently discussed the fact that there are a number of autoimmune and immuno-regulatory abnormalities associated with HAE. To be on the safe side, I’d advise against being a bone marrow donor unless it was for a related person with a life-threatening condition. In this case, the risk would be outweighed by the potential benefit.

Dr. C: With regard to blood donation there may be some concern as well if a patient is on some of the plasma derived (although very safe) products such as Cinryze or Berinert.

Dr. Z: There are two requirements for blood donation: first, that it be safe for the donor; and second, that it not pose a risk for the recipient. As long as a HAE patient is not having an attack at the time of the donation, being a blood donor should be safe. It’s much harder to address the second half of the requirements. The rules are that people cannot donate blood within a year of receiving a blood transfusion. Cinryze and Berinert are much safer than blood transfusions, but the blood bank may still be wary. In general, people with “immune” diseases are not supposed to donate blood. Whether this would include HAE, is again a matter of interpretation. In my experience, the blood bank will usually not accept a HAE patient as a blood donor.

Dr. C: Leaving the blood bank aside, do you think that donating C1 inhibitor deficient blood could cause a problem for the recipient?

Dr. Z: We know that the contact system (the kallikrein-kinin system that generates bradykinin) is unstable in patients with HAE. I would expect that the process of drawing blood and then re-infusing it would almost certainly activate the contact system and this could have some deleterious effects. So, I would also come down on the side of not donating.

Dr. C: Thank you Bruce. I hope that we have provided some helpful insights on this topic for our HAE community. I will look forward to our next discussion of ‘The Question of the Week’.

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