Hi all. My 85yo Dad is now in a critical condition. He has rampant hemalosis, hemoglobin of 70, extreme fatigue and as of today, excrutiating pain in his back, groin and scrotum, with black urine. Scan ruled out kidney stones, doctors unsure of the cause. 2 doses of Obinutuzimab and Chlorambucil plus 2 more treatments have not done a thing for his CLL. Awaiting results of bone marrow biopsy. He has had 10 transfusions in 2 weeks, just keeping him out of the danger zone. My question is this: Could the multiple transfusions have caused the hemalosis? His blood type is B positive, relatively rare, and I have read that unless the red blood cells are cross-matched exactly with his, (which they were not until yesterday) introduced antibodies will target the persons existing red blood cells, 'clump' them together and destroy them, causing a domino effect. Does anyone know if this is true? After 5 years of w&w, this has all happened so quickly. Three weeks ago he was playing golf. Thanks in advance.
Desperate for answers please...: Hi all. My 85yo... - CLL Support
Desperate for answers please...
I'm sorry to hear your 85 year old Dad has uncontrolled AIHA. There are many causes of AIHA but as he has CLL then the most likely cause is his CLL dysregulated immune system which is making antibodies to his red cells. Blood transfusion is an important supportive treatment and life saving when the Haemolysis is severe so it's good he's getting that. Blood group B positive is uncommon but not rare and the blood bank will have stocks or can order it in for him. Even if they don't have it then you Dad can receive blood from a group O person quite safely and that is the commonest blood group in most parts of the world. Blood transfusion departments go to great lengths to make sure the blood is safe and it will be as compatible as it can be with your Dad.
The difficulty with the antibodies made by his CLL is that they are often not very specific and so can attack and then haemolyse the transfused red cells as well. That's why multiple transfusions are needed over and over again until the haemolysis is under control.
Transfusions can support him for a while but it's important to stop the Haemolysis. Treatment is usually an effective method of stopping haemolysis but, from what you say, it doesn't seem to have stopped it yet for your Dad. Steroids such as methylprednisolone are often used in high doses in the first instance and are often effective and you don't say if this has been tried.
Another useful treatment is ivig or the transfusion of normal antibodies from donors into your Dad. This is also an effective treatment for many people although several transfusions are often needed to get good control.
Treating the CLL, which is the root cause of his haemolysis, is the best way to get long term control and the doctors may need to consider using one or both the two methods I've mentioned above or changing his treatment by possibly adding Venetoclax if that is available in your country and he is fit enough.
It's important that he is supported with lots of fluids to keep his kidneys safe and also folic acid to support his own production of red blood cells as he can quickly run out of the vitamins needed.
Hope this helps
Jackie
Oh thank you soo much Jackie. We are in Australia. Venetoclax has just become available to us! All of what you said makes sense. I just thought that maybe the slightly unmatched transfusions could have caused the AIHA, as he didn't have it before those (??). He is on 50 mg prednisone daily. I will make sure he has folic acid. Will discuss the IVIG, thanks. I am so worried about the black urine and pain. Hopefully they can sort it out on further investigation. Thanks for your input and advice. Tracy
The black urine is caused by the pigments in the broken down red cells. It's possible to develop gall stones with haemolytic anaemia, although that usually takes some time, and that could cause pain. It sounds as though they have checked his kidneys thoroughly which is good.Wishing you both all the very best Tracy, let us know how he is.
Jackie
I had haemolysis in April this year and spent 4 weeks in hospital, i am 79, once diagnosed treatment was Rituximab and :Methylprednisolone by infusion, they cross matched my blood every day in case they needed to give me a blood transfusion, but wanted to avoid it if possible but thankfully the treatment was all that was needed.Hope your Dad's condition will improve soon.
Thank you so much. Take care. Tracy 🌷
I just noticed your Dad is on 50mg of Steroids, at the beginning I was on 70mg but that didn’t have any effect on the Haemolysis, then they gave me 1,500 mg of methylprednisolone and Rituximab after that haemoglobin slowly increased.
He likely has AIHA as others have suggested. It can be urgently managed with high dose steroids, IVIG, rituximab and other therapies. This is usually a fixable problem with the right aggressive medical care directed at the AIHA first and the CLL secondarily.
Thank you, I spoke to his heamatologist today, he is calling it 'rampant hemolysis', and won't budge from the Chlorambucil and Obinutuzamab treatment, saying it will work if we give it time. Gosh, hope my Dad can hang on.🥺
Obinutuzumab will probably help, though rituximab is better studied. Chlorambucil can itself cause hemolysis. I know of no role of it in treating AIHA. I would recommend getting a 2nd opinion about stopping it. IVIG and high dose steroids work quickly to start hemolysis. It sounds as if he is hoping to control the anemia by controlling the CLL. That is not the primary standard of care Get him to budge or get another doctor involved. This is urgent.
Hi. Last night Dad had an episode where he passed out. He now has fluid in lungs, as he drank too much water while on a diuretic and has become oversaturated(?). Another transfusion today as rbc was at 70. Doc says 'these things happen'. He is due for Chlorambucil and Obinutuzimab Round 2 next week. We are scared to move him to Sydney now, but are so scared for his survival.
Totally agree he needs Rituxan stat!
Thank you....I don't think we have that one on the Australian PBS as yet 😟
Obinutuzumab is a better, second generation version of Rituxan/Rituximab/Mabthera.
Neil
Yes You are right Neil but they should be able to get Rituxan because it has been around so many years my husband had an excellent response from it
Based on this report on the use of Obinutuzumab/Gazyva on CLL patients with AIHA: tandfonline.com/doi/abs/10.... , I would presume that Obinutuzumab would work equally well if not better than Rituxan.
"Patients with CLL and AIHA receiving obinutuzumab showed continuous and stable increase in hemoglobin levels concomitantly with decrease in parameters of hemolysis."
Worldwide, Obinutuzumab has been slow to replace Rituxan despite proving to be a more effective CLL treatment. Rituxan has been in use for a few decades.
In Australia, Rituxan is available for the treatment of CLL in combination (FCR) and as a monotherapy for Follicular Lymphoma.
Neil
It seems strange why Gazyva has been so slow to replace rituximab , I assume cost must be a large factor. ??
Gazyva has proven to be more effective in numerous studies
You are absolutely right and I reread that he was already receiving Gazyva with Venclexta so perhaps they have ruled it out. I do believe I remember Jeff saying Gazyva worked for him when Rituxan did not.
Sorry to hear about your dad.
Has AIHA been confirmed (I assume he has specialist CLL docs - this is important as he is rare). Others above have mentioned high dose of steroids - in UK the dose is 1mg/kg/day, so exact dose depends upon his weight. The high dose (4-6 weeks in UK) should stop it.
This is a high dose and so there can be side-effects (possibly made worse by his age, so maybe they will go lower?). However the shock of the dose should stop the AIHA in its tracks.
Then gradually he will need to be weaned off the steroids - issue is when you get to end of weaning (ie off the steroids)....in 1 out of 3 cases, the AIHA comes back (in my case there was no sign of it coming back until either down to 5mg/day (I started at 80) or even totally off the steroids). However, in your dad's case if this happens, they might be able to keep him on a lower steroid dose longer term. In younger patients (I was 53) they don't like to do this as very long term steroid use has unwanted side-effects.
Hi there, yes, it is AIHA. He has a haematologist attending, who seems a little blase. He's on 50mg of prednisone daily.
has he had Ivig?
I don't know why they are not administering Rituxan stat
Hi there. Yes, I asked the doctor, Dad has had IVIG a few times. He also says Dad has to wait for the next treatment, Chlorambucil and Obinutuzimab (Gazyva) on 8 Dec. He thinks the 3rd treatment will work. We are nervous. We asked to change to the new drug Venetoclax, but he wont budge, and Dad is too weak to move elsewhere. Such a dilemma. Thanks for your input.
I am so concerned for your Dad!! Like Dr. Koffman said above, treatment for CLL is not the treatment for AIHA! He needs treatment now not a week from now! I hope he is continuing to receive blood transfusions to keep his levels somewhat up.. If he is short of breath due to the anemia and finds it hard to breathe, ask them if he can get oxygen. I did that when my husband had such a hard time taking a deep breath when the hemoglobin was below 7or8. For some reason I guess this is not protocol, but once they started it he felt much better. And my husband is a nonsmoker so can't blame it on COPD or that. All the best to you and your father.
Hello, thank you for your concern. Yes, he is short of breath and on oxygen. They are giving him transfusions almost every day, but the rbc keeps going back down the next day. Not sure what to do... I have tried to be assertive in telling them about this group's great advice, but they keep saying that Chlorambucil and Obinutuzamab is a great treatment, it will turn around soon and we just have to wait till the 8th. So frustrating!😪
Truly chemotherapy is no longer recommended for people over 80. I am so sorry you are going through this. Is there a way to get him transferred to another facility? Husband was the same, transfusions only lasted a day and right back down. Never went up much more than one point. He had it back in late June. He dropped from 14.5 hemoglobin to 2 weeks later with the dark urine etc. down to 9.1 and the next day 8.5 and finally down at lowest 6. It was very scary, he is 81 so close to your dad's age. The latest protocols say that adding chlorambucil to Gazyva is not going to give a better outcome and in my husbands case it lowered his white counts to really low levels and ANC was below 1. By the 4th day when I could not get him to a hospital where he could get the Rituxan infusions - and remember, this was going backwards since Rituxan is an older drug than Gazyva and he had been treated for the CLL with Gazyva and chlorambucil like your father. Rituxan turned things around within a day! If you have to maybe you could print these comments out and show them to the doctors. I am willing to send you my husbands medical records with the treatment summary. He has continued to improve monthly to where today his hemoglobin was back to 14.7. This should not be life threatening because there is treatment and I don't know why they are not giving it now. I am pasting this in to show you the treatment plan: His care at MCI was resumed at the time of urgent admission this
month to West Kendall Hospital with a hemoglobin of
approximately 6 g, white count up to about 60,000, with normal
platelet count
At West Kendall he was started on Solu-Medrol as well as given IVIG
No significant improvement in counts, and patient transferred to
Baptist Hospital where he received split dose Rituxan
Counts improved and patient was able to be discharged on 60 mg of
prednisone
In summary:
Solu-Medrol between June 13 and June 19, prednisone started on
June 19 and continued up until today's office visit/total 9 days
steroids
IVIG given on June 15
Rituxan given on June 17 and 18/currently day 9
During recent hospital admission, received a total of 2 units packed
red blood cells
Hemoglobin stabilized and improved, white count decreased during
this course of therapy
Hope this helps!
You need a specialist CLL haematologist......not a generalist haematologist, especially if they are blasé. CLL requires a specialist in CLL as it is such a hetrogeneous condition - and only 1 in 10 get AIHA on top of the CLL. So he is rare. A generalist is not enough.
I know, you are right. I am trying hard to rattle chains, but we are in small town Port Macquarie where there are just 3 haematologists to share in the whole mid Coast region, let alone a CLL specialist. He says he is consulting with Sydney, I really hope so. I will keep trying, thanks so much for your support.
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