CLL Support Association
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Help! Newly diagnosed 31yr old very confused.

Hi All!

My 31 yr old brother was diagnosed with CLL this week. He is not symptomatic but has/had extremely swollen lymph nodes for several years (at least 4-5yrs) he did not have medical insurance and it wasn't bothersome so he never went to the doctor until I got him insurance recently. I am a physician assistant student with a good basis of medical knowledge yet still I am finding his lab work difficult to interpret. I am interested to know more about the flow cytometry results, his CD19,CD5&Abs Cnt, &Kappa are all high, and his Tcell markers are a mix of low and high values. His WBC is 88,000 with a 95%lymphocyte count. I am interested in knowing more about the flow cytometry and what these values mean. I read about the ZAP-70 and IVGH, are these included in a flow cytometry? Or what test would you ask to be done? What does the FISH test give information on? The chromosomal abnormalities associated with this condition? My brother doesn't understand medical talk to begin with and this news has left him very confused. I am hoping to help him understand and choose the best treatment in his case. I know CLL is not typically a young person disease, so there is likely some chromosomal abnormality. Any advice/experiences on testing and treatments would be very helpful. His doctor mentioned possibly immunoglobulin protein treatment? but I am wondering why not chemo given his young age and no co-morbidities. I intend to set him up with a specialist in CLL who can answer all his questions. Obviously, with him being so young we are very confused on why this happened. We have no known family history. Again, appreciate any help, suggestions, feedback. If you need specific values of labs I can provide them.

Thanks again!

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Wow that is young, but I know a girl that had CLL at age 19, had a stem cell transplant, and just recently had her second child... 😀

I would get to a major CLL research center, ASAP and then they will help get the ducks in a row. If you mention your brother's location, we may be able to suggest a few research hospitals and doctors...

Flow cytometry gives B cell markers which form a barcode that says CLL or something else. IGHV direct mutation test, is not used too much clinically, because CD38 and ZAP70 give reasonable indications of mutated or unmutated.

FISH panels show genetic deletions, additions and translocations, they are done more frequently now just before treatment, because they can change over time... CLL was thought to be a static cancer, but in the last few years, we have learned it is very dynamic, always changing...

I would certainly think about an allogeneic stem cell transplant, due to his age. The treatment carries some risk but can be curative.

Certainly treatment options are expanding and we are nearly able to control CLL throughout someone's normal lifespan in many cases, so while there is never a good time to get CLL, things are moving froward rapidly, and your brother has AGE on is side... a very important prognostic factor, often overlooked.

All the best

~chris

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Sorry to hear you have joined the family. The strangest thing about getting your head round CLL (and I was diagnosed just a few months ago at 46) is that treatment is not always recommended. Indeed treatment too early has been shown to increase mortality (I,e, make people die sooner!). So the other poster is right that it is important to get yourself to a top centre asap and get their advice. My understanding is that the diagnosis flow is as follows:

Raise lymphocyte count which look suspicious under microscope suggests the diagnosis

Flow Cytometry confirms it

Rate of increase in white cell count and the presence of other signs and symptoms seem to be the main indicators for treatment

Mutation analysis helps to indicate the likelihood or otherwise of the CLL progressing fast.

So it is a case of getting an assessment of staging (how many lymph nodes and where, etc) and also the mutations, but ultimately watching and waiting to know if treatment is required. Oh, that and having immunisations like pneumococcus and avoiding live vaccines.

Its tough. But you are not alone. And many live for a long time with this illness before requiring treatment. Good luck!

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Good points - particularly about the importance of avoiding treatment until absolutely required.

There can be many triggers initiating diagnosis, as this past survey shows: healthunlocked.com/cllsuppo...

Yes, smear cells - broken CLL cells, are a fairly strong indication that someone may have CLL. In my case it was a falling neutrophil count that led to my diagnosis (bone marrow infiltration had reduced my ability to make these infection fighting white blood cells), but my blood test results regularly come with the comment "Smear cells present".

Good to see your encouraging update and to hear that you are still in watch and wait - long may it last.

Neil

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Hi thank you so much for your reply. We are located in Connecticut and definitely willing to go anywhere in the state with good care. I read about a women at Yale who specializes in CLL and is doing a lot of research. Fossa or something like that was her name. I was thinking of calling over there unless anyone has some suggestions. Unfortunately, he has Medicaid insurance which will not allow him to get care out of our state (I already looked into Mayoclinic who wouldn't see him and he would be considered a self pay) so any help is really appreciated with this. Thanks again for taking the time to help me and my family!

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Here is the doc at Yale you mentioned ~ good luck! Definitely become established with a CLL specialist ~ many standard hem/onc folks don't understand how complex and highly individualized this disease is.

Francine Foss, MD, Yale Cancer Center, New Haven

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Thank you we called her today! The hem/onc doctor he first saw and got the diagnosis from is at smilow also but in Waterbury instead of new haven like Foss ..... I am hoping they will not give a hard time about asking for a second opinion from a doc in the same practice but differrnt locations but I did hear she is very good. Hoping she will see him. Thank you again!

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Hi Jade

I was diagnosed last October and had to begin treatment immediately, I was age 38 and was told this was very rare as was a disease usually for people in later life.I have been a member on this site since and it seems to be more and more common in younger people now.

It's a complex Illness but very treatable, my personal recommendation wouldn't be to follow the chemotherapy route unless he has specific markers which could in some cases entail a very long remission if not a cure, the reason I say that is it's a disease of the immune system so my logic is why damage yourself further when there are many new softer treatments available which could offer a better outcome in the long term.

In answer to your question why your brother has this illness early in life is a complex question to answer. Specialists are beginning to realise this isn't a one treatment fits all disease and are running different tests and different therapies for many individuals in order to treat it and it's probably the same for the cause, it could be stress lifestyle a weakness from early life damage from another illness or a number of other possibilities.

One thing which you may find comforting is this is probably the best time to be diagnosed with it with so much research and new promising treatments available with more daily in the pipeline that under the right team there's nothing stopping people from living a full life.

If possible I would suggest getting yourself a cll specialist as they will be up to speed with all the latest therapies and advise, there are also many knowledgable people on this site to answer any questions you might have and to guide you.

Good luck and let us know how you get on

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Hi Lewis, thanks so much for your reply and advice. Would you mind sharing what kind of treatment route you went and why? What was the reason you started treatments immediately? My brother has very swollen nodes in his neck, armpit, and groin, but no abnormalities in his RBC's yet. I really know nothing about this and what to gain as much knowledge as possible. I really appreciate any information you can provide.

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Hi Jade -

I'm sorry to hear about your brother's diagnosis. He's fortunate to have you as an advocate - especially as you're in medical training!

I'm sorry to hear he's been diagnosed so young, as well. That's a blow. We expect our bodies to carry us through with good health for many decades and it's frightening to get a diagnosis of "incurable cancer" at a young age. Feels like being cheated. But those of us diagnosed young can reasonably expect a cure within our lifetimes. Maybe not tomorrow, and maybe not without side effects, but a treatment to de-fang this cancer.

As to why . . . it's such a mystery. I'm relatively young for this disease also and I have an autoimmune disease (psoriasis, since I was 5). Autoimmune disease can prematurely age the immune system with respect to mutations and errors, because it makes our white blood cell machinery work much harder than needed and mistakes can be made along the way. I don't know if your brother has anything similar, just relaying my experience. But something (even just random chance) caused a transcription error resulting in the original cancer cell rather than the normal one that was supposed to have been born. And now we live with it.

Anyways, it sounds like you're going to get him to a CLL specialist and it sounds like you want to go to the doctor armed with good questions. I'm linking a couple resources I found really useful - the first one is about understanding blood tests (with a CLL focus) cllsupport.org.uk/cll-sll/s... and at the bottom there's a link to formatted spreadsheets if you want to track the results of your brother's tests over time.

The other one is from Khan Academy, you may like it especially since you're in medical training. It describes the basics of CLL cellular biology in a few minutes. Khan Academy has a whole series on blood disease that's wonderful. healthunlocked.com/cllsuppo...

Take your time with learning - it's a huge amount of information and don't feel badly if you need some time to absorb it, both intellectually and emotionally. It's typically a very slow moving cancer, so you'll likely have time to learn about it before your brother will need to make any decisions.

Best to you both. You are an amazing person to be an advocate for him! <3 This webpage is a great forum for community support, you aren't alone.

Marie

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I will definitely be looking into all of these I spent so many hours doing google searches of articles trying to find stuff about CLL and lab results. Thank you so much!

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Hi everyone! I cannot thank you enough for all your responses these have really given myself and my mom a glimmer of hope. She will be joining also after hearing your helpful responses. Thank you again!

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Cll society web site helps. Good info.

Get a 2nd opinion from a Cll specialists .

Don't get any treatment to quickly. Look closely at look tirn side effects.

Uc San Diego an other centers avoid FCR chemo therapy.

Keep learning.

Hoffy.

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Thank you so much! I will keep trying to learn!!

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Hi Jade,

These papers may help you with understanding your brother's bloodwork. What caught my attention was the omission of CD23 from your post. Given the young age of your brother for CLL DX you need to be sure his diagnosis is accurate. CLL can co-exist with other blood cancers or can be misdiagnosed as CLL when actually it is MCL (Mantle Cell Lymphoma).

Title of paper: "Prognostic importance of flow cytometric immunophenotyping of 540 consecutive patients with B-cell chronic lymphocytic leukemia | Blood Journal"

bloodjournal.org/content/78...

Excerpt from a Journal “Blood” guidelines for diagnosis and treatment paper.

"CLL cells coexpress the T-cell antigen CD5 and B-cell surface antigens CD19, CD20, and CD23. The levels of surface immuno- globulin, CD20, and CD79b are characteristically low compared with those found on normal B cells.8,9 Each clone of leukemia cells is restricted to expression of either kappa or lambda immunoglobu- lin light chains. In contrast, B-cell PLL cells do not express CD5 in half of the cases, and typically express high levels of CD20 and surface Ig.10 In addition, the leukemia cells of mantle cell lymphoma, despite also expressing B-cell surface antigens and CD5, generally do not express CD23.

bloodjournal.org/content/bl...

Connecticut is where I was raised and depending on where in the State, say South West, you could take the Merit Parkway into NY to consult with Dr. Furman or his associates at the Weill Cornell Cancer Center which is a pioneer in non chemo agents or if in the North Eastern part of the State you would be closer to Dana Farber another source of CLL specialists.

Believe me when I tell you that the inconvenience of travel to get expert advice in managing your brother's CLL is critical for optimum outcome. Even if you use a more local oncologist an opinion from a specialist is well advised.

WWW

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Hi there.... Finally yes thank you I have been looking for resources to understand the flow cytometry results and I was having SO much trouble I will def go thru your comment with his labs when I have a chance. Maybe I can upload his labs to and get some feedback bc like I said this is way above my knowledge scope but I am trying. I would love to get him I to a specialist at either of the places you mentioned but unfortunatly (and fortunately lol) he has state medicaid insurance which they will not accept out of our state I dont think. Maybe I can convince him to spend the few hundred dollars just to consult with someone you mentioned and then he can have treatment if necessary in CT where its covered. Anyway he will be seeing a specialist at Yale soon but again I cant thank you enough for all the info and taking the time out to explain to me I really appreciate it.

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Also while I have it on my mind I read about ZAP-70, and IVGH do they go by a different name on a flow cytometry? Would it not be included in a typical flow cytometry? I heard those can give prognostic info also. Thanks again!

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Hi there so I posted his labs (page 3 has the Flow) and now have a few mins to look over your very in depth and super helpful post.

His labs show:

1. high CD19&CD5

2. Normal range for CD20&CD23 from what I see?

3. Kappa light chains with CD19 are high while, lambda light chains with CD19 are low.

There's some other abnormalities listed in the T cells and NK cells but im still not sure I understand enough to formulate any questions without wasting your time or engage in a productive conversation bc you seem to know alot.... so I am going to look into the link you posted and see if I can get a better understanding of what all these markers point too in this case, and like you said see if they could point to something else like the mantle cell also. I very much appreciate your post i dont fully understand yet but I will with a little time. Whats ironic is my last semester back at PA school starts on Tuesday and it will be heme/onc and blood cancers so all of this info is really helpful and also giving me a head start on my studies!! Thanks again.

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IGHV mutation status testing is a separate test and is useful in the sense that once done and established it will not change. ZAP-70 is a difficult test to get right and is not very useful without methylation data to go with its percent of expression. ZAP-70 is difficult for the reason that it is expressed on T-cells as well as some CLL B-cells. Your brother might have trouble getting approval for either test. FISH however is essential data to have if chemo regimens are considered for therapy options.

Kinase inhibitors like Ibrutinib and idelalisib or their 2nd generation alternatives work very well in both indolent and aggressive CLL profiled patients. Venetoclax, a Bcl-2 inhibitor, is another recent drug that works well on all varieties of CLL if considering drug treatments. Therapy decisions are likely not for now but given the age of your brother there is much to sort out only after proper testing is done and what options are available to him that could be obtained in CT.

WWW

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Hi everyone I tried to post his lab work under this thread but I don't think it worked there are 5 pages. I will try to figure out how to get them into here but they are all labeled "lab page 1,2,3,4,5" if I can't get them in here and you are interested to take a look. Any info is helpful. Thank you all so much again!

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CLL Society web site mentioned by Hoffy is cllsociety.org - good information and a list of reliable diagnosis specific links .

CLLTopics has good information about Flow Cytometry, the FISH test (though anything about prognostic markers is dated, as new treatments have changed things considerably), and mutational status.

The LLS has good printed information and telephone counselors who are very helpful. Both links on the Society page.

It would be great if your brother could get a second opinion from Dana Farber - Jennifer Brown or a member of her group, or Dr. Furman or Dr. Lamanna in NYC. Medicaid may allow a single consult out of state. Smilow seems to be putting a lot into their hematology program. My cousin is seen there for follicular lymphoma (another blood cancer). I just got his "begging message" about supporting him in the fundraising bike ride that they do every year.

Where are you in Connecticut? My first teaching job was in Meriden and I had an aunt and uncle (and their families) in Storrs and another uncle in Cheshire (I'm in Southern CA). I still have cousins in Storrs and Groton.

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Hi thanks so much for your reply. He will see the specialist at smilow in New Haven on Friday. We were raised in Cheshire. I currently live in New Haven. Nice to hear from people familar with the area. After the visit on Friday I will see if Medicaid would allow a consult in NY like u said. Would love for him to get a second opinion from Dana Farber if possible... And will keep the list of people you mentioned if they allow him to be seen. Really appreciate the info and time!

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My cousin, Mike Kennedy will be riding in the Smilow Closer to Free fundraising ride Saturday. You might want to check it out. It might be something you or other family members would be interested in doing in the future if you have any hard core athletes. or you could support one of the riders you know. Mike would love to have you on his list if you don't know anyone and want to get involved. I asked him who his doctor is at Smilow and who his doctor would recommend for CLL - the price of my donation! LOL! I suspect it will be the one your brother is going to see, but it's nice to have others agree with your choice.

I wonder whether your parents or you knew my aunt and uncle, Bill (William E) and Ruth (Riggs) Kennedy or any of their children in Cheshire - Kathleen (Kat), Bill, Chris, Mike, and Sansy.

Pat

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The names do sound familar .... Ill have to try and jog my memory to see if I can figure it out. Normally I would love to get involved. I used to work at Smilow and do these activities often. Unfortunatly I am in PA school right now and a bit strapped for extra time lol but I def appreciate the info and you asking him for his advice. We actually saw the new doc at smilow today!!! His name is Godfried Von Keudell MD (my spelling may be off). We had called to book with Dr. Foss but were told she isnt specializing in CLL right now, that Dr. G Von Keudell is who we needed to see. Anyway, saw him today and really could not be happier with the choice. We spent 2+hrs at his office and with him answering every question we could think of and explaining everything about this condition. I was really so impressed and thankful we found him after leaving there today and my brother, my mom and myself feel alot better about the diagnoses and future. I cant thank you enough for taking the time out to help me and I would be interested to know what Mike says about a good doctor there still. In the future maybe next year when things are not so hectic I would love to go to the event. Thanks again!!!

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UPDATE: hi everyone.... Just wanted to post an update on my brother and say thank you to everyone for all the info.... Really helped us have intelligent and productive conversations with our new doctor at Yale today.

We saw the new doc. He was amazing. We spent 2+hrs explaining everything anyone ever wanted to know about CLL. He answered all our questions and I feel my brother now has the information and confidence in his doctor to proceed with treatment. The doctor is reccomending FCR (I believe) chemo. He will have 6 rounds. He ordered a bunch of tests like the FISH and some others ( I forget the names) but he is giving my brother a month to think it over and return back hopefully ready to start treatment which he is reccomending right now bc of his age, and the magnitude of lymph node swelling he has in neck, axilla, and groin. Interestingly, and looking for any feedback from you guys on this. I started doing some digging into our fathers side of the family. We dont know him and have never had a relationship...so I didnt know the family history from that side but felt like I needed to. Anyway, our father (mine and my brothers) apparently has CLL, and his sister (our aunt) also has CLL... But no other members have it. I dont have more details then that right now but I plan to find out more like age of diagnoses (although I believe was 50/60yrs), chromosomal abnormalities etc as soon as I can. I couldnt find a ton on inheritence of CLL just that 10% of people with CLL have some blood cancer in their family. Thought it was odd that both him and his sister have it, and now my brother (at 32yrs old-young for this diagnosis) has it. Unfortunately, I tracked down this info after our apt with his great new doctor today but I will obviously bring this up at the next meeting. Any one with any studies/info/personal history anything on inheritence would be so so appreciated. Again thanks to everyone for all the help thus far!!!

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CLLCanada (Chris) has posted about this. Unfortunately the search engine on this site is not great and I didn't come up with his posts. Hopefully he will see your question. I'm glad that your brother's appointment went so well. He will want to know his FISH results before committing to FCR, but barring any problems there, FCR would be the choice of several top doctors, given his age.

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