Hi All,
CLL diagnosed in 2014 and HLH on top of that in 2018. HLH makes CLL look like a cold it's nasty and I thank God for every day that I'm still here and as of yet not needed a transplant. I'm now 43 years of age. Is anyone out there in the same boat?
Have a great weekend all! 😊
Sorry, I don't recall anyone mentioning HLH before, so here from en.wikipedia.org/wiki/Hemop... is the condensed version:
Hemophagocytic lymphohistiocytosis/syndrome (HLH) is a life threatening uncommon hematologic disorder/disease of severe hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and macrophages, characterised by proliferation of morphologically benign lymphocytes and macrophages that secrete high amounts of inflammatory cytokines. It is classified as one of the cytokine storm syndromes. There are inherited and non-inherited (acquired) causes of hemophagocytic lymphohistiocytosis (HLH).
Sounds like extremely exacerbated CLL fatigue! I hope my explanation boosts your replies,
Neil
Cheers mate 👍🏻
What would be the non inherited causes?
Do not share your HLH, but want to send prayers for relief to you. Sandra🙏🙂
It sounds nasty. Can it be controlled/cured? I do hope so, that is a very unfair shake of the dice.Cx
HLH is a new one on me. Are you still on Ibrutinib? Which is given to patients after CAR-T to suppress cytokine release syndrome.
Venetoclax for nearly 2 years now
Hi Shirty,
I was not familiar with HLH and just did a little additional reading on it. I am so sorry you were diagnosed with CLL in your 30s and also have to contend with this additional diagnosis. I wanted to send good vibes your way and hope that if and when transplant becomes necessary that you will have an excellent response. Please keep us posted on how you are doing. I will be thinking about you!
Warm regards,
Nan
Shirty78,
Sorry to hear you are dealing with this and glad that you are doing well. HLH is treatable, even curable, if caught early. It's so important to have a team who are specialists...or at least one HLH specialist leading the team. Since many organ systems can be involved there might be multiple specialties involved. Care can vary according to what is perceived to be a precipitant event...or if there is a familial predisposition. There is conflicting literature and opinion as to whether ibrutinib can help or even, conversely, trigger HLH...so important to have that HLH specialist.
Thank you!
Hi, Shirty78
Wow! Finally a compatriot. You hit the nail right on the head. Nasty is exactly how I describe HLH. I have faced HLH twice. From what I have learned about HLH, we are very lucky to be alive.
My first time was in late 2016 at age 58 while I was being treated for CLL as part of an acalabrutinib trial. HLH nearly killed me. My liver and kidneys started to fail. I spent three weeks in hospital. Not knowing what else to do the doctors put me on CR as a last-ditch attempt to keep me alive. I responded quickly, but it was another 2 weeks before I was released. When I left the hospital, I could barely stand up or walk without assistance and I had neuropathy in both my feet. The doctors followed up with 5 cycles of FCR (They excluded the F in my first round because of concern for my kidneys).
After almost 2 years I recovered enough to return to work in the fall of 2018 but after a few months back, in early 2019, I was in hospital again with HLH. This time the doctors suspected HLH immediately and got it under control while they looked for an underlying cause. They diagnosed Richter's transformation. The doctors started RCHOP immediately on diagnosis. I left the hospital shortly after treatment. But this time I was given a grim prognosis and have returned to work.
I am currently in remission. A stem cell transplant was recommended. However, a complete match could not be found. The surgeon felt my heart had become a little too damaged to risk a haploid SCT.
So I am still here 5 years later living one day at time. It's been two years since I finished RCHOP. My CBC is much better than it ever was between my CLL diagnosis in 2009 and the RCHOP I received in 2019. Unfortunately, despite a consistent two-year effort so far of regular physical activity, I have been unable to restore my strength and stamina to the level I reached in 2018.
I appreciate hearing about your experience with HLH. Perhaps you could share it with me directly off the list. HLH is very rare in adults and surviving it is also rare. We HLH survivors need to stick together. Perhaps we should get ourselves t-shirts attesting to that fact. Or maybe even start a support group for HLHers. I found it very hard to deal with the devastation that HLH brought to me and could have benefited from sharing experiences with others in the same situation.
Cheers and thanks very much for posting.
Yes we both are very rare in a bad but also a good way.For me FCR brought it on in 2018.
Hospitalised for 4 months with all sorts of issues couldn't get out, transplant patients came and went I was still there, no transplant! 🤣
An English doc realised my issue week and half in.. dexys and etoposide was the treatment.
Twice I have done it now.
Strange thing I got back to work pretty quickly and still working a physical job now.
Bloods are all good ferretin is still higher than ild like it at 6000 but it's better than 360,000 that's right not a typo.
Nurses are still amazed I'm here and tell me regularly when I head back for monthly venesections.
Thank you for your comment!
We could start a group but it would prob just be the 2 of us mate!
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