Following my (currently) monthly haematology consultation, I am now beginning to understand the AHIA (autoimmune) complication...looks like 10% to 20% of us get this at sometime). Symptoms of AIHA include in isolated fall in haemoglobin, positive DAT (what's that? - I will explain), rise in reticulocytes (AIHA risk factors at at end of this post). AIHA can be caused by CLL (it can also be sometimes caused by other things). Mentally I am now considering it as a separate complication, with separate treatment regimes (which do NOT count as a CLL treatment): the key I have learned is that some of the symptoms of AIHA can be confused with progressing CLL.......the CLL might not be progressing after all.
In my case, when diagnosed in Jan, the CLL was clearly BINET stage A (only the spleen is currently effected) and RAI stage II (again spleen growth puts me here). What I did not know at the time was a Coombs test also showed a positive DAT. This means effectively my red blood cells are being coated and there is thus a risk that my body (spleen) is fooled into thinking they are a foreign body and starts disposing of them. This is the cause of increased risk of AIHA. My understanding is that the Coombs test is a standard test in CLL diagnosis process (in UK at least). So our DAT score (positive, or negative) is a bit of information we should be asking for! Should also the CLLSA blood test guidance mention it/explain it?
In the first half of this year, my spleen grew to over 8cm larger than it should have been, haemoglobin dropped (to where I was beginning to think the CLL had moved to Stage III on RAI...so were things getting worse?). The drop in haemoglobin was compensated by a large rise in reticulates. So my spleen had indeed decided the red blood cells were foreign and grew to cope with their mass destruction: but a healthy bone marrow increased red cell production to largely compensate. So actually, knowing my bone marrow is healthy is good news on the CLL front!
My drop in haemoglobin is ENTIRELY caused by the AIHA: there is no reason to believe that the CLL is contributing and so I remain Stage II. So the underlying CLL remains stable: again good news that I only really understood yesterday.
The treatment for AIHA has been a very high dose of steroids. I think this lowers immune response and the spleen thus no longer thinks the red blood cells are foreign (the spleen sets a tougher test to decide is a body is foreign: the coated red blood cells now get through the test as not-foreign). During high steroid doses, my haemoglobin increased and reticulates dropped to healthy levels (as in CLLSA blood test results guidance). The steroids also dramatically increased all white blood cell counts (lymphosite and neutrophil)...so the lymphocyte doubling time measure is currently meaningless and not relevant to understanding CLL progression - it is caused only by the steroids.
I am now in final stages of coming off the steroids and haemoglobin currently remains ok. My understanding is that as I have had a short-sharp bust of steroids (over 3 months), there should be no complications of coming off that people who are on long-term steroids get (where the body's natural production stops during steroid treatment and then fails to re-start after treatment).
If I am lucky (around 66% of people - next 6 to 12 months will tell), my spleen will somehow (no-one knows why) have been re-programmed to recognise my coated red blood cells at actually part of me (ie not a foreign body) and so not re-embark on a destruction policy. If I am unlucky, there might be a second treatment option (however, if steroids don't do it, I am in a 'difficult to treat' category, so the second option I don't think has huge success rates), then/or my spleen might be to be removed to stop the AHIA. Spleen removal is not without complications, but it might have to happen.
So what are the risk factors for AIHA. they are positive DAT (I only found out that is me yesterday), advanced CLL (not me), high white blood cell count (I was only just over normal counts, so not me), older age (not me), gender (me, as male), poor prognostic markers (unknown for me, as FISH test only done when looking at CLL treatments) and initiating (CLL) treatment. So looking at that list, I was thinking I only had one (male) with two unknowns (DAT and poor CLL prognostic markers).....which was obviously was worrying as had I got AIHA because of poor prognostic CLL markers? Well I don't yet know, but at least it is possibly that being male with a positive DAT is enough....so I can remain positive!
Overall I have learned:
1 - know your DAT...it has probably been measured. A positive means increased risk your red cells will be seen as foreign and thus get destoyed
2 - AIHA can give measurements that can be confused with CLL progression: however, they are caused by the AIHA, alone NOT the CLL so don't get concerned/mix it up with CLL progression!
Hope this helps others facing the AIHA complication!