acquired angioedema

[hertler13]

Has anyone been diagnosed with AAE caused by their CLL.? I was diagnosed with CLL in 2008, but the cause of all the swelling attacks I was having was not figured out until 2013. I have been through two 6 month treatments with FCR, the first because the CLL had reached the treatment stage and the second mainly to try and control the acquired angioedema attacks. I am currently on 280mg of Imbruvica daily and Orladeyo for the AAE. I am 68 years old. Thanks

[AussieNeil Partner Administrator]

Sorry, but with only one related post: healthunlocked.com/cllsuppo... acquired angioedema must be pretty rare in our community.

Neil

[UKwoman]

Yes, I also have AAE which is thought to be caused by my CLL. I was diagnosed with CLL in 2006 and then I started having symptoms of angioedema in 2019. My GP didn’t initially think it was related, but the haematologist did and I was sent to see an allergy specialist. I was twice sent to hospital as the swelling became so bad very quickly including the side of my face, lips and throat. Since seeing the allergy specialist, I am taking 1 x 180mg fexofenadine tablet a day, and can take up to 4 a day if necessary. They told me after six months it would normally have controlled it, but as they believe it is related to the CLL I take it every day. My haematologist said he believed if I eventually have CLL treatment, this would probably sort out the problem. My age is now 67, so we have a similar profile! My CLL progresses very slowly, although I am now anaemic and being more closely monitored. My only other symptoms caused by CLL is warts over both hands. These simply will not disappear despite trying many treatments, and in fact I have had them surgically removed twice but they come back within six months. Not a lot of fun.

[hertler13 in reply to UKwoman]

UK woman, yes we do have similar profiles. I had my first swelling (hand) long before I was even aware that I had CLL. That was 2005 and I continued to have more and more swells of the hands, feet, intestinal tract and face, which are the scariest. I had my first 6 month long treatent of chemotherapy for the CLL in 2012 with FCR .The swelling attacks seemed to lessen after that, but still continued and no doctor that I saw could determine what was causing them. In 2013 I had a face swelling so severe that in the emergency room I lost my airway and had to be intubated. I spent 4 days in the CCU with a respirator breathing for me until the swelling went down. They tried all the usual drugs that I would receive during a swelling attack, IV steroids, antihistamines etc, but the swellings usually went down on their own. My oncologist at the time said "We have to get to the bottom of these swelling attacks or they are going to take your life" and he sent me to a local immunologist, which in fact, I believe, did save my life. As fate would have it he had seen these same kind of swelling attacks during his residency that was occurring in an entire family. There are 2 main types of angioedema that cause these problems. HAE, that gets passed down by families and AAE that is usually caused by a Bcell malfunction, in our case that is CLL. The problem is in the complement system and your doctor needs to know what blood work to order to see what your levels are. My levels were so low they were not able to be measured. My CLL specialist in Boston had 1 other patient with this condition and suggested that I go thru an another aggressive 6 month round of chemo to get the CLL to a point where the complement system could start functioning again. I did that and was swell free for 5 years. In 2019 my I started having swells again, so I was put on Imbruvica and also take an oral medication for the AAE.

I would suggest finding a doctor that is familiar with the complement system and having the blood work done.

[UKwoman]

Thank you so much for this information. I did have a C4 blood test and was told I must never take CE inhibitor class of medications and also no nonsterodial anti-inflammatory drugs. However, now I hear how desperately ill you have been, I will go back and ask for more blood work to be done.