My dad was diagnosed with CLL in January. obviously we were shocked but between the doctors and everything we read, we found some comfort in knowing it was pretty manageable.
Since then, he's had 2 follow ups, one in may, and one in sept. I believe he was initially diagnosed at 14k WBC or ALC? Im not quite sure, it's a tough topic and I dont like to pry with him about it.
in june, it was up to the mid 30s, I believe it was 34 or maybe 36. In early September, just over 2 months later, it was 67. so he started treatment.
I know he is del17p+, tp53 not mutated, and IGV? mutated.
I understand del-17+ is the worst prognosis factor. That was a blow for us. But more recently, I have been panicing about the time to doubling. His has more than quadrupled, possibly even 6 folded (since I cant remember if he was diagnosed at 11 or 14) in less than 9 months.
That sounds like its pretty aggressive, right?
he started calquence about 2 weeks ago. but my fear is that it might hold things off for 2,3 years, then come back even faster than the scary rate it was already moving. If thats the case, at this rate, he would be in the 200k range in a year and some change
Does anyone have a similar story? That it doubled like every 6-8 weeks after diagnosis with del17p? i guess Im trying to benchmark what to expect. I am very worried, he was only 62 when he was diagnosed, just turned 63 less than 2 months ago. he's pretty broken up about it, we all are.
Ive seen some stories on here about people with del17p on calquence for like 8 years and counting. i am hoping thats how this will go, of course. i am wondering if those for whom that played out that way for....was your doubling time that aggressive too? he basically had 0 W&W.
I mean he was technically on W&W between jan and sept, but my understanding is, W&W is the period where you are measuring doubling time. it would take at least 3 tests to get an idea of its "speed." and this to me seems....bad. really bad.
he is with a stanford CLL specialist, so he is in good hands and I think she is very knowledgeable. but she cant control things like speed.
my sister has a young son, only 6 months. over the weekend, my dad was holding him . I dont think he thought I could hear, but he said in a little baby voice, "you're not going to remember me."
it really made me so deeply sad.
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Glasscheetah
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A couple of things to consider, from a CLLer with a doubling rate of 3 months and a 300 ALC at treatment start:
1) ALC count, by itself, is only a general indicator of CLL progression. There are many patients with ALC > 100 who do not require treatment. There are some with ALC 50 who do. The driver for CLL treatment (and, by extension, serious CLL health issues) is not ALC. It's impact on spleen, lymph, and other circulatory systems... and how those impacts actually create a substantial issue.
2) ALC doubling rate is an indicator of CLL development speed, sure. But it's not an indicator of CLL severity. Some people have problematic mutations with slow doubling rates. Some have "plain vanilla" CLL with high doubling rates.
3) Treatments can be effective regardless. I've been in clinical trial for 18 months now and the treatment has been effective. It's not to MRD/remission yet, but it looks like it is going to get there around the two-year mark.
4) Will the past doubling rate hold for the next W&W / treatment cycle? Who knows? I don't believe there's been a substantive finding one way or the other.
I'm honestly a little surprised in your dad's case that a) treatment was started just on doubling rate, and b) that they opted for acalabrutinib (Calquence) which is not a fixed duration treatment. At his age, FCR chemotherapy is possible, and could lead to a durable remission. There are downsides to FCR but it might be worth the risk in this situation. I was looking at that route myself but opted for clinical trial instead.
When asking about fast doubling rates, I've generally gotten vague answers from some fairly well-regarded CLL experts. I'm not sure how much is known at this point.
But I'd consider maybe getting to a specialist for a second opinion. CLLsociety.org can help point to US-based options.
Having the "fast version" of the "good cancer" is sort of a double whammy. You get all the shock of the C word while also having an experience that is different than the majority of CLLers and this community. It is tough psychological territory. Good luck sorting through it with your dad.
I have a bit more info in my profile and in this post about my reasoning on treatment options. healthunlocked.com/cllsuppo...
Thank you for the detailed reply. This is my fear. his is not moving slow, and is the high-risk variety. at this rate, he'd be in the six-figure counts within the first year, event thought it was caught pretty early (either 11 or 14k counts, I cant remember).
I think it was the combo of the doubling rate (which would be like yours. every 3 months or so) and the del-17p confirmation. my understanding is that it means its progressing quickly AND its of the high risk variety...a bad combination.
he is currently seeing 2 doctors. his primary and a Stanford CLL specialist
The stanford doctor ran the genetic testing in Sept.
When she told us the results (the 17p situation), she said we should start treatment.
It all is happening so fast. we were told at time of DX that it could be in watch and wait for years.
and of course, thats what we were hoping for.
but looks like thats not how its playing out for us, since its more than doubled in every appointment since.
I have read that doubling time is a progonsitc factor, which makes sense. slower growth would naturally be a better things. From what I've read, people whose doubling rate is more than a year have a better outlook. With his being every 2-3 months, it sounds to me like its...moving very, very quickly.
he does have swollen lymph nodes, and he was moved from stage 0 to 1 in the last appointment.
From what I have read, it seems that though in relapse/refractory tend to also have faster doubling rates and are more likely to have high-risk mutations than those who havent been treated yet. My understanding was it is believe that chemo may actually cause these mutations. dep-17p occurs pre treatment somewhere between 5-10% of the time, but between 30-50% of the time in relapse/refractory cases.
i hope you achieve MRD soon. and that it never comes back
FCR chemotherapy doesn't work when del(17p). The chemo disrupts the DNA copying during cell division. 17p has the gatekeeper that orders the faulty cell to die.
Well, keep in mind that doubling speed does not correlate to treatment effectiveness/resistance. And there are multiple effective treatment options.
Look at it this way: even with doubling every three months, if you are starting from a low base, it will hold back for a while. If you get CLL back down to MRD (Minimum Residual Disease) levels, that means less than 1 in 100000 cells are CLL cells. Even if it doubles every three months you have several years before it's an issue again. And many treatments today are achieving that kind of result.
Hello Glasscheetah, its understandable that you are concerned about your dad. I have a few observations as a cll patient currently on calquence.
I am not sure where you came up with the calquence only working for 2 or 3 years. Calquence is a better btk drug than ibrutinib, but we have more data on ibrutinib. As a firstline treatment for cll, ibrutinib can work for ten years or more, even for some folks with 17p cll. Calquence may do even better. Most with 17p make it at least 6 or 7 years before progressing on btk drugs as a first treatment for their cll. There are good second treatment options now for those who do progress with their cll. Five and ten years out, we will almost certainly have new options.
Most folks with 17p are TP53 deleted and have unmutated ighv. You report that your dad does not have a TP53 deletion and is ighv mutated. That is not typical, but it would certainly help your dad's prognosis if you are correct he has no TP53 mutations and is ighv mutated. Either way, he should do well on calquence.
My wbc count was moving slowly on me and then went into hyperdrive, I was at 150k one week and 200k the next. I don't think you can read a whole into a fast doubling time so far as meaning he wont do well on his cll med options. I expect over the next few months you will see your dad's labs improve dramatically while on calquence.
Its natural to worry about the future, but better to enjoy the now. I think just with the current meds, not even including the ones that will come along, that your dad can go on for many years with a good quality of life. Good luck, you're a good son to be worried about your pop and active in his care.
yes, thank you for your response. I am confident in the muted IGHV and non-mutated tp53, although I am a little confused on that front, as it seems to me the del-17p IS a mutation of tp53, but Im just learning about all of this now, so I'm sure theere's things I dont fully understand yet.
Initially, I thought "mutated" would be a bad thing, and I was quite confused that unmuted was actually worse (almost feel like there should be a separate term for that "status" considering in general, cancer is a mutation, so mutations seem "bad" overall).
Now my understanding is that it has to do with the "patient 0" cell that divided into the first cancerous cell, with unmuted being a younger, more stem cell-like cell, and mutated being like...an older cell that had lost some of its "stem cell superpowers" as it does in a natural lifecycle progression.
And even though regular stem cells are good, we dont want that stem cell "superpower" to be part of the cancerous cell's geneic make up. I figured unmutated it meant the cancerous cells were more robust and harder to "kill" or contain, and probably divided faster, since they are like....cancerous semi stem cells. That we don't want the cancerous cells to be stong and hearty. I figured that would also influence LTDs, that unmuted cancerous cells would theoretically divide faster due to still having some of those stem cell like qualities, but my dads are mutated and dividing rapidly, it seems.
I am extrapolating the 2-3 year estimation of time btks will work for him based on the median I found in studies. I was looking at data about median times of PFS specifically for people with del17p, and it seems to fall somewhere between 45-60 months. But I'm being more on the cynical side, mostly because of the LDT. after all, that does mean that 50% of people did NOT make it to that median....and if his are proliferating at this rate, he might not be on the upper end of that scale.
To your point, the doctor also said it was rare to only have the del-17p but not the tp53 mutation, but that seems to be his case. I understand its pretty rare to have del17p prior to any treatment, as well.
Still, most of the research I found on it made it seem like del 17p/tp53 were sort of the "same bucket" in terms of prognosis — that is, high risk. I have read about how he is a "single hit" mutation, versus double, or even triple hit (like del-17p AND the tp53 mutation, etc, with each mutation compounding adverse outcome probabilities.) I do understand his mutated IGHV and unmuted tp53 should improve prognosis probabilities,
The LDT is still really alarming to me. It seems to me like...the muted IGHV should mean a slower LDT, but from all the anecdotal stories I've read in CLL forums, seems like his is among the fastest. A lot of others are saying they'd have a 15%, 50% increase per year...so his 2-4 month LTD is extra concerning to me.
The learning curve is steep for cll markers and there is a great deal of nuance. I am not an expert, but my understanding is as follows.
When one has 17p deleted cll it means they have some missing genetic material on the “p” arm of the 17th chromosome. The reason 17p cll can be challenging is that the TP53 gene, which is on the 17p arm, is usually deleted or mutated. That is bad because the TP53 gene is an impt cancer fighting gene, like a self repair kit. It would be unusual for someone with 17p cll to have an intact TP53 gene, but it happens. I would think the prognosis is better with a functional TP53 gene, if you are understanding the reports correctly.
IGHV mutation status is an entirely different thing. If the mother cell of all the bad cll cells had gone through certain normal mutational changes before it went bad, one is said to be IGHV mutated, which is a very good marker. It would also be unusual, but not impossible, for your dad to have 17p cll and have mutated IGHV. The mutated ighv would still be of benefit, despite the 17p.
It’s possible to have an intact TP53 gene that later loses function. IGHV status, on the other hand, very rarely changes. I am only guessing here, but if your dad does have an intact TP53 gene, his mutated IGHV status could have contributed to that benefit. Why IGHV is such an impt predictor is not very clear, there are different theories.
When you look at progression statistics, it is impt to look at the patient population studied. People who take drugs like ibrutinib and calquence as a first treatment (treatment naive) have considerably longer progression free survival than people who have had other Cll treatments. The last study I saw on treatment naive 17p folks starting on ibrutinib had 60% still doing well 6 years out. Calquence might do better. If your dad has an intact TP53 gene and has mutated ighv cll, I would easily put him in the group that gets a longer benefit with treatment.
If your dad suddenly had an increase in his wbc doubling, I do not think you will find the answer as to why. It happens to a lot of us, me included.
This is just me, but having gone down the marker rabbit hole for a couple years after my diagnosis, in retrospect I wish I had worried less about them. I have good makers, but for some reason my cll acted aggressive anyway. We have many people with poor markers doing great.
Has your dad’s dr suggested his doubling time will shorten his progression free survival? I’ve not heard that. Even so it’s just a guess, no one knows. And I’m not trying to sugarcoat things, but I think your dad can live a long time with 17p cll just based on treatments we have today. For many on here, thats the game, cobble treatments together until the next one comes along.
thank you for this detailed reply. I am still missing something though. If the tp53 gene is on the 17p alelle, would the tp53 also be deleted if that allele was deleted? I thought it was the other way around.
I know an allele is half a chromosome, one of the two strands that makes up the doulbe helix. so maybe he's just "lucky" (if you want to call it that) and his tp53 gene is all "coded" on the other side of that chromosome.
that was what he said. and yes, the specialist said it was very unusual to have del-17p but a normal tp53. at the same time, its pretty rare to have either or even both for people who are untreated.
man, 60% is....not a number i'm comfortable with at all. That...sucks.
but i don't have any say in it, and i guess no one else does either :/
HiI don't have Del 17p , however I am the next level below in terms of prognosis. I was diagnosed at advanced stage 4 at 56. WBC was 3.6 , Plts 52 and HB 80. I had 100% marrow infiltration and multiple internal swollen lymph nodes 2 as big as cricket ball near my heart. I went straight to a blood transfusion and Obinituzumab + Ibrutinib. I would in UMRD within 243 days. It is now 4 yrs since my diagnosis and i am on Acalabeutinib twice daily. I have monthly bloods and 6 monthly Flow Checks... my remission is holding. I have odd IVIG top ups and GCSF in my fridge just in case Neuts drop. I am now 60 full time dad to my 7 and 5 yr olds and live full pre CLL and Pre Covid life style. I know not exactly the same as your dad but I hope it shows that drugs are great. Also because 1 in 200 people get CLL, Pharma pump billions into drugs as it a good earner for them.
also, I cant help but wonder. what triggered the escalation? I know the best you could do is speculate. but was there something going on when your counts' started accerlating?
These BTKis are excellent. I am unmutated and have been on Calquence for 4 years. In all that time my bloods have been normal and I feel great. I'm on a pause at the moment as they sort out a few joint problems but expect to be back on again for another couple of years before I'll need another treatment. I am sure your dad will do well. I know he will be very worried but we are in the era of great treatments.
I had 6 years on WW and was progressing at a "normal' rate. However in October 2019, I caught in Madrid a virus that made me extremely ill for a month. (?covid? - who knows) After that my lymphocite count went from 77k to 240k within an 11months and I had landed at stage 3 with almost 100% infiltration in bone marrow. I can't tell you if I was 17p del but despite the acceleration Calquence knocked my CLL count into the ball park. My consultant also told me to take Vit D3 and I know there has been a lot of discussion about CLL and low levels of Vit D3 but personally speaking I think it has made a difference.
This has come up with my MCL...mutation is a loaded word for TP-53. In some studies, it includes mutated and deleted folks. In others, it's just mutated and deleted are their own category.
Is your dad TP-53 NORMAL or is he TP-53 DELETED?
As for treatment, it seems early, but if the lymphs were becoming a problem (or growing rapidly/profusely), it would probably not be too early.
I am pretty sure its tp53 normal. the doctor commented that it was rare to have del-17p and not have the tp53 mutation. but im not sure if the del17p is on the tp53 or not....sounds like...no?
i found this aspect confusing, because everything I read about del17p seems to also be talking about tp53.
If you use the edit function, you can choose to show your post only to this community (vs the entire internet/world) - it's a selection at the bottom under the post.
Folks tend to put more personal info out when it's limited to just here.
And yes, it would possible to be normal TP53...it's just really not the norm...
At the moment the whole internet can find and see this post. People are more open when a post is locked to be seen "community only". Below your first post, there is a "More V", select "Edit", at the bottom of the edit page there is a box for "share", change it from "anyone" to "community only", then "post" it again.
Acalabrutinib is a good treatment for those that have 17p/TP53 aberrations. These drugs don't tend to change mutational status of the CLL, that was something that chemo did as it selected for what were higher risk sub-populations. Acalabrutinib seems to be able attack all of the higher risk sub-populations.
IgVH mutated with del(17p) is about 1% of patients at treatment, without TP53mut is even fewer >0.2% (0/440).
His blood lymphs will rise dramatically over the first few weeks as the B-cells are evicted from lymph nodes.
Read my profile, I was originally thought to have an acute leukemia. I have the "bad" del 17p and my variant usually doubles in about 4 months when lymph counts are high & it's active. I started treatment within 6 months of diagnosis, and that was back in spring 2011.
Oh, my WBC went from "normal" to over 50,000 within 6 months. I had my yearly physical in the spring, things seemed fine. I was sick late December. My bone marrow (tested by biopsy since originally they were working up an acute process) was almost pure white instead of spongey red.
He's getting treated, they are on top of it. It's hard, but please try not to worry. It's not the markers, it's how he responds to treatment. I was expected to die without a stem cell transplant & I'm still here.
wow, so your case is very similar to his. I just didn't see anyone in forums who's numbers were doubling so quickly. thank you for sharing.
I was just thinking if the meds work for a few years and he becomes desensitized to them...this thing will probably come roaring back quickly, based on these short doubling intervals.
i know there's nothing we can do but see how it goes. its just all very fresh...we just got back the third test, giving us an idea of the LDT and thats where we learned about del-17p.
We got used to the idea of the leukemia, even optimistic about it. the doctor didnt seem to worried initially, possibly because the WBC wasn't very high at time of Dx. I cant remember, but with 11 or 14k, so presumably, caught it early.
We thought he wouldn't need treatment for many years. I mean i knew it was a possibility, but I just....assumed he'd fall in that category, to the point that I wasn't so worried about it. he's otherwise a pretty robust guy, and on the younger end of the spectrum (I read average age of diagnosis is 71, and he was Dx'ed at 62). so its been shocking that it escalated so quickly.
I really hope he responds to treatment as well as you have. And that the meds just keep working for both of you — forever.
I was 52 at diagnosis. And I am taking the stance more like, "beat it down a while, then treat when it rises again." If one gets mmmm 2? years in between time limited treatments, a number of clinicians will repeat the same regimen. It's not necessarily "one runs out of treatments." And a number of people get many years on the same one without resistance. So IDK where you are getting "it only works for 2-3 years" for the Calquence. Some may get resistance, some won't. No way to predict ahead of time.
IME it's a crapshoot if one responds, for how long, and whether or not one gets few or numerous side effects. Docs make great educated guesses based on recent trial information, but there's no guarantee. So don't panic if this one doesn't seem to be optimal for him, another one likely will. Some people start treatment, have few problems, and sail along for years. And some don't. But even traditionally hard to treat people like me, get some remissions in there among the failures.
We all react to a "cancer diagnosis" but need to remember this particular one is included in a number of those considered "chronic", not "acute." "Chronic" diseases can and do get so severe they impact people; some people with diabetes or heart disease are very ill. But quite a few aren't. So hang in there :). Until you have learned to dig deep on the Internet at reputable medical sites for the most recent information, the top things you are finding on search engines are likely older data. Statistics and outcomes are changing, to the point where quite a few of us are expected to die "with" this disease, not "because" of it.
Did your Dad’s doctor tell you why they picked acalabrutinib instead of Venetoclax? Two friends of mine and I are in our 50s and had very fast growing CLL. All of us are on Venetoclax. The team of CLL specialists at Stanford are among the best. Your Dad is in good hands.
My wife at 67 was diagnosed with 17p TP53 in 2015. The internet gave her 2 years to live. In a year of watch and worry or watch and wait she was finally put on Imbruvica late in 2017. Her lymph nodes on her neck and shoulder blade disappeared in about a week and a half. Her white blood count went from about 20,000 to 98,000 as the lymph nodes emptied out into a blood stream. After about two months they put her on Venetoclax only and her blood work fell from 98,000 to 5000 in about 4 weeks and stayed there for about 10 more months until 2019 so they gave her a Venetoclax vacation and her blood work remained normal for 37 months....with no meds.
When they began to climb to 13,000 in 2023 they put her on Acalabrutinib for less than a year and then she took another vacation from all meds and has been in the normal range for about a year. These new oral agents (pills) are astounding. I like to think of it as playing whack a mole and the moles are losing. After over 9 years she doesn't really worry about it much anymore.
Just a quick comment about the doubling. Think of the words horsepower and torque, horsepower is how fast you're traveling toward a wall, torque, on the other hand, is how hard you hit the wall.
So maybe the time to act is sooner, but the therapies you receive are effective. Additionally, if one doesn't work there are other medicines and procedures that may be more effective.
I know it isn't anything solid you can feel relieved over, but time may show you that the management is still going to be worth it. Let it show you, don't rush to see what hasn't happened yet.😌🌻
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Doc says it's usual to start treatment with doubling of WCC
Afraid of Low blood counts
Change in my CLL. Faster doubling time
Altered perception of time
