CLL Support Association

CLL / P17 with Mutation

My sister in law who is 60 yrs. old has been diagnosed with CLL / P17 deletion with mutation. She has BWC of 213,000 and swollen lymph nodes. She was put on Hydrea that brought it down to 177,000 then they added Imbruvica. Hopefully this will bring it down until she can have a complete stem cell transplant. The doctor said Chemo will not work with this condition. Does anyone out there have any info for this? Thanks for all of your help.

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Hydroxycarbamide is an unusual drug to use in CLL... is your sister in law being treated at a large research medical centre, or a community clinic?

17p deleted patients do not do well on chemo immunotherapy, this is true... Imbruvica (ibrutinib) or newer drugs in clinical trials are her best options, prior to transplant.

Are they recommending and auto transplant, or allogeneic?

~chris

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The Hydrea was used to bring her WBC down enough to take the Imbruvica. Hopefully they will take her off that soon. At first she only had 2 twice a day for 5 days and her WBC went up but then they double it 4 twice a day then it went from 213,000 to 173,000 in three days. That was this passed week. It will be a complete stem cell transplant if the meds work!!

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It has now been 7 months. What is happening? And what hospital is P17 deletion is a not to be fooled around with - it is aggressive and deadly. Right now an Non-Myloablative (Mini) Haplo Allogeneic PBSCT is one of her only options. The chemo drugs really do not work. Venclexta works. And you can use it alone! Do not add a chemo because the chemo does not work. Once you get the cancer cells in the marrow as close to -0- as possible (Not >20%) do the transplant. The key is doing it at a place like Johns Hopkins that will use a half match and they know how to control GVHD well. Not many centers will do a half match because they do not have the skill. You can live a long time with the P13 deletion, as noted by someone later in this thread, especially if you are mutated. But you cannot live that long with a P17 deletion. It is NASTY!!!! And I know from personal experience. Car-T can help to get you to transplant but ultimately when you get to near -0- you need to go to transplant. There are really not plans C,D, and Es. Trust me I know. My transplant is in 2 weeks and I am so thankful that I am finally at a place to have the transplant. My son is my donor, yes, if done at a place like Hopkins you do not need a full match. Get second and third opinions, but only by one of the TOP specialists. And, these oncologists at the Top know each other and each others work. Good luck.

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This is an old post (8 months). One downside to this site is that posts don't get jumped to the top when new comments are made. You may want to introduce yourself and post your information in a new post. Check the bottom of the post for community / everyone. Locked posts (community only) keep your information private as well as that of anyone responding, usually resulting in more responses.

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There are excellent new therapies for 17p- patients. Ibrutinib is one and venetoclax (Venclexta) is another. There are others and new treatments with immunotherapy drugs continue to emerge. I agree that you should be seen in a center wherein they have CLL specialists. Transplant is a risky option and I would thin that one would exhaust newer immune drugs before considering that option.

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According to the doctor the Imbruvica will only work for a while and a complete stem cell transplant will be her only option to live. That is because of the P17 mutation.

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I agree with the other replies concerned for your sister in law regarding the bone marrow transplant recommendation, as the use of these is decreasing with better, non-chemo treatments. She should seek a second opinion from a specialist CLL centre. The risks associated with bone marrow transplants increase with age, a cure isn't guaranteed and lifelong side effects from the transplant are a concern. One well recognised CLL specialist, Dr Rick Furman, (New York-Cornell) said on the CLL/SLL Groups.io forum on January 9th this year "For the patients with deletion 11q or 17p, venetoclax plus ibrutinib would likely be the way of the future. I am assuming acalabrutinib will look very similar to ibrutinib in the long run with regard to efficacy."

Acalabrutinib is a second generation version of Ibrutinib with less side effects and is currently being assessed in a clinical trial.

Neil

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Just so she is getting opinion from major CLL center. Is she?

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Yes

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Have a look at this article: ericll.org/pages/publicatio...

The issue is that newer therapies have not yet been indoctrinated into the decision tree leading to stem cell transplant. Therefore one should get an opinion from a major center involved in clinical trials with acalbrutinib/ibrutinib, idelalisib, venetoclax, etc.

I am lucky enough to be a physician at Stanford where I am confident that my treatment is state-of-the-art. I am certain there are comparable centers in the UK or wherever you live.

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You don't say where your sister in law lives. If you give a general idea someone here may be able to help her to find a doctor more knowledgeable about CLL. The treatment approach she is getting is unusual for CLL and there are many CLL patients with 17p who do well on the newer non chemo drugs like Imbruvica. I would certainly encourage her to get an opinion from a CLL specialist before planning to go to transplant. It really is a last resort approach these days with so many new, promising meds.

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I am a patient and though a little younger, I am also 17p deleted (with a bunch of other complications), so I have a similar profile. Please, please get a second opinion. I am not a doctor and I don't know her particular situation but I am doing to OSU and am getting best-in-class treatments and I can tell you what they tell me which in a nutshell is a stem cell transplant for me is an end of the line last resort. I am already on ibrutinib and venetoclax and I've asked about what Plan B (and Plan C) are if this doesn't work, and neither involve a stem cell transplant (or CAR-T for that matter). Maybe these are 5th alternatives, and there are new drug discoveries all the time so I'm hopeful I don't get there.

It is true that I get tested for ibrutinib resistance frequently, and that some 17p patients grow resistant to in as little as 18 months, but not everyone does. Some are doing great for years. Ibrutinib does make your WBC go up for the first month or two (especially if you have bulky nodes to clear out) but that turns around.

Can't help but mention I assume that they just make the diagnosis, because any specialist would have told you to start treatment for 17p sooner rather than later even if there are no major symptoms typically used to tell when it's time to treat people without 17p because things can get out of control quickly. My WBC went from ~60K - 90K in about 10 weeks, and I went from noticeable to huge lymph nodes in 6 months. If she has been under a doctor's care for some time, treatment should have probably already started.

Once WBC goes down sufficiently (or once if ibrutinib stops working), venetoclax is an option. You have to ramp up carefully to avoid tumor lysis, but that's because it works so well.

I don't know where you are in the world and stem cell transplants have been lifesaving for many on this forum, but the mortality rate is high (it keeps changing, but double digit for sure) and your life is on hold for many months while you are in the hospital and recovering in the hospital.

If I get to the point where my doctor says this is where I need to go, I will walk down the plank, but in my case, that isn't happening for awhile and a specialist may give me far better options than you are considering now.

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My sister in law never went to the doctor at all. She fell at her work and broke her foot last year and had to go. Her hip kept hurting so when they X-ray it they saw all the lymph nodes were swollen so its went from there. This has all happen in the last two months so its not been long since she was diagnose... She feels good about her doctor. She is a specialties on this and seems very knowledgeable!! The doctor did tell her the stem cell transplant will be way down the road. I'm just concern about her being in denial. She thinks all will be OK and this will soon be over but I know its just begun. Thanks for all of your info I appreciate all the help we can get to help her. How long have you had your CLL and did they give you any life expectancy? We are new to this to and are very concerned. Her husband my brother has only been dead for three years he died of a different cancer at the age of 58. Thanks again!!

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Just seeing this post 7 months later...I was diagnosed 12/2015 and started my trial 6/2016 which ended 9/2016 and I was at MRD+ both BMB and blood at .01%. Still on ibrutinib. WBC still normal. There was no guidance about life expectancy, and the way I look at it, in 2010, the average was something like 32 months, but I have met people with 17p deletions diagnosed 10 years ago who are still doing fine. I was given no guarantees about life expectancy, but when I had my first consult, Dr. Byrd said that at my age, we needed a plan that would go for 40, not 4 years. I did ask what would happen if I relapsed, and he rattled off a list of other drug options he would even think about CAR-T or a stem cell transplant. I have made the choice to find a great advisor (Dr. Byrd) and to listen to him. I'm not in denial, and it doesn't sound like your sister-in-law is either.

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Very good information. I am 53 yrs old and 17P deleted. I started treatment on a clinical trial in San Diego Jan 12th, 2017. On Imbruvica so far but start Venetoclax this Thursday!! So far by Imbruvica has worked well with not too bad of side effects.

Just curious what do they say are plans B and C?

Is the goal of your trial to get to MRD Negative and then get off all drugs for a while until needed again? That I think is the hope so that drug resistance does not occur,

thanks

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There is only A and B right now or at least that's all they have talk about. A is to get your WBC down and B is to get a complete stem cell transplant!! I do know the meds only work for a while then your count starts going back up!! What did they tell you? How high did your WBC go up?

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My WBC got up to 90K but my nodes were getting very big and painful. Also my LDH was getting high at 1600. They are not clear on plans B or C yet. There are many new drugs coming out as well.

My WBC was 9K last month.

Is the hope of your trial to get to MRD negative and then get off all drugs for a while until it comes back? This way we avoid drug resistance. I am told that is what is done in CML many times- but I might not have heard it correctly.

Were you on Gazyva as well?

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Right now she is only on Imbruvica. She never went to the doctor for anything. This was found due to her falling and breaking her ankle. Her WBC got up to 213,000. It was down to 176, 000 when she was taking Hydrea with the other med. She had to come off of Hydrea because of side effects. She has only took this two meds for the two weeks. She just found out she had CLL about 4 weeks ago.

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Sorry for the late reply. The goal was to get off the drugs and many in the trial got to MRD-. I didn't, and they kept me on ibrutinib. I probably would have been kept on it even with MRD- because the tests aren't that sensitive and I am 17p deleted.

Plan B might be Lenalidomide, currently being studied at OSU as a treatment option for CLL and other leukemias and CAR-T might be Plan C.

This is a fairly digestible article about new novel agents and drugs in the pipeline:

ncbi.nlm.nih.gov/pmc/articl...

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There is no plan b and c. When your bone marrow gets close to 0% cancer you need to go to transplant with a p17 deletion. You really do not have an option. Have you read all the literature. Venetoclax works. It only lasts on average 1-2 years and there really is not anything better out there right now. So if this gets you to where you need to be take advantage of it. Go to the best transplant center in the country and give yourself the option of a cure. Right now there is no other cure and once the treatments fails, morbidity is almost certain. I have had to face this and am thankful that I am now ready for a transplant. Honestly, most p17 deletions never make it to transplant. I hope you get to be one of them - and are cured!

Best,

CLL patient with 17p deletion.

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I'm still hopeful that the combo of obinutuzumab/venetoclax/ibrutinib gives me enough runway to get to the next breakthrough. If my doctor recommends a stem cell transplant, that is what I will do (and I will do what it takes to have it done at the best center), but at this point, it's not a recommendation he is ready to make. I hope your transplant is very successful!

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14 years since diagnosis and doing well. What is your sister in law's general location. If she had to get this diagnosis her timing is good. Treatments today were unheard of when I was diagnosed.

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Do you have p17 deletion @ 14 years?

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No. 13q, but my case is very atypical, as the CLL presents in the kidneys. How is your sister in law doing? I know patients with 17 p who have been diagnosed for almost as long who are doing very well on Ibrutinib and some who have moved on to Venetoclax.

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Thank you for your response.

Am new to this site and perhaps am jumping in on someone else's thread?

I "myself" am recently diagnosed with CLL (within the year). WBC was climbing rapidly. In June Oncologist ran FISH and p17- discovered. Within weeks was started on Imbruvica. Have been taking Imbruvica a little less than four weeks. WBC nearly doubled to 100,000 as of yesterday.

While it appears new treatments are on the horizon, I am attempting to get a realistic prognosis with what's available today. When I saw your "14 years" I felt compelled to reach out. Am not familiar with 13q.

Thank you for your time and response. It is much appreciated

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Sue - It didn't connect that this is an old thread. Your response triggered an e mail that it was here. I would start a new post about your situation and any questions you have. One downside to this format is that threads don't bounce to the top when new comments are made, so most people will not see your information in this thread. I'm sure you will get helpful insights about both 17p and Ibrutinib. It is normal for the WBC to shoot up before starting down.

When you post it's a good idea to lock your post to community only. You are likely to get more responses to a locked post, as the original post controls the privacy of any answers.

The ACOR CLL list currently has a discussion about longest survivors. My 14 looks like not much compared to some who have posted.

A good resource is cllsociety.org - lots of good information and a list of links to reliable, CLL specific resources (including ACOR).

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Thank you so much for taking the time to respond. I am more than a little slow on tech knowledge. So I will try your suggestions

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No one is slower than I am when it's about anything tech! Looking forward to your introduction. Where are you being treated?

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Loma Linda Campus @ Murietta, Southern California. My Oncologis is Dr. Schinke

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I had 13q deletion for over 25 years with a mutated status. When my CLL mutated to p17 deletion survival did not look good. Thank you Venetoclax. Currently, my marrow has <0.4% cancer, have no palpable nodes and my Stem Cell transplant is in 2 weeks. This is my only option. P17 is horrible! I have not heard of any p17 deletion patients that have survived for many years once treatment is needed. Research studies have shown that once treatment is needed, response to standard first-line agents and regimens is very poor and response duration is short. It is aggressive and mean! Right now from all that I have read and learned is that transplant is the only way to get rid of it. I sincerely hope your CLL does not mutate to include the p17 deletion! Good luck with your journey.

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She lives in Ga.

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Second opinions are very important. This is true for any major decision in life.

Good doctors many times want you to get a second opinion,

Even if you plan not to work with the 2nd doctor it sheds light from a different angle on the situation,

Hoffy

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I know someone with 17 P who has all the bad indicators and has been going for over 12 years and has been doing fine on improve a car for the last four .

Hopefully carT or another breakthrough will help us in the long run .

Hoffy

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