As some of you have posted about previously, today I also entered the NIH natural history study for CLL. The visit was good, efficient and pretty painless. Getting on the campus was straightforward only requiring a driver's license and a pre-registration with the CLL team that was seen by security. Admissions was quick and travel reimbursement was painless. Phlebotomy was next and they snagged 13 vials of blood. Next stop was the 7th floor to meet the clinical team. After the standard vitals I was met by one of the fellows who spent about 30 minutes reviewing my previous blood work as well as family cancer history, occupation, etc. and today's lab results. To my pleasant surprise my blood numbers were even better than my draw in June with my ALC dropping to 1.91 k/mcl with all other numbers in the normal range. LDH (200 U/L) and Beta2 (2.4mg/L) were also down from June. At this point the doctor stressed I had SLL (something I already knew), but she added "you don't have Leukemia you have Lymphoma." I'm not sure if she did that for me or for her, but I found it interesting because we do often view it as both when it sometimes is clearly one or the other. She ran off and briefed the team and then all 6 of them returned for a Q&A session. I really didn't have that many questions (most have been answered here), but I did ask about the grim reaper Richters because I'm Trisomy12, Unmutated-IGHV with a Notch 1 mutation. After the expected "rare event" and heterogeneous disease discussion they did say they would like to get one more blood test which I'll do at MSK in a couple weeks because the NIH lab had already closed. They want to dig into my IGHV to see if I have IGHV 4-39. Not sure if that's a mutation or deletion, but thanks to a Dr. Jennifer Brown YouTube video I know it's a suspect driver of RT.
Anyway, a good visit, glad I did it and they'll have me back for a follow up in a year.
Bigfoot
Written by
BigfootT
To view profiles and participate in discussions please or .
You and I are twins. I am Trisomy 12, Notch 1, unmutated. I was heading to NIH in May, but didn’t get to go because my lymphocytes grew from 65 in February to 130 in May. I started V+O in the hospital the same day I was supposed to be at my study.
What did NIH say about our Richter’s risk? I have had multiple conversations about this topic with my CLL specialist. He said my odds are very low (less than 1% in next five years) because I don’t have a complex karyotype and/or TP53/17. Your IGVH test result should list your type of mutation. I have VH-169 which is most common.
My doctor has shown my multiple studies that show that Trisomy 12 responds better to treatments than any other type of mutation including no mutation. He said, “Trisomy 12 unmutated typically grows quicker and is easier to kill than any other type of CLL.”
Penny, that level of germ line is not in my lab results so that's why he wants another test. I THINK if you don't have 4-39 then stop worrying. If you do....maybe it's time for some early intervention to reduce clonal evolution chances.
Jeff, I appreciate the reply. Dr. Wiestner was great and recommended the deeper germ line testing for 4-39. The doctor who made the lymphoma vice leukemia comment was a young fellow. I honestly think she said it more for herself than me. Thanks to your CLL for dummies and all of Neil's posts I was well prepare for the 1 on 1 conversation. She actually chuckled and said, "wow you could pass your medical boards on this disease". She wouldn't get it but I wanted to say you should read Cajunjeff's posts.😄
The best SLL/CLL explanation I've seen is that most recent Dr. Furman Q&A video. He says prior to 2004 they were 2 distinct diseases and a person could have a lymph node biopsy and BMB done on the same day and get 2 different diagnosis from the same pathologist. At that time they acknowledged they were the same cells and the same disease, but they evidently couldn't come up with a new name so we get the slash.
Strictly speaking, CLL/SLL (chronic lymphocytic leukaemia/small lymphocytic lymphoma) is the most commonly diagnosed adult Non-Hodgkin's Lymphoma, so your NIH doctor was absolutely correct They became a common disease with the 1994 World Health Organization classification update of haematolymphoid tumours and lymphoid neoplasms.
Our pinned post from 10 years ago on the difference between CLL and SLL healthunlocked.com/cllsuppo... quotes Dr Furman "The confusion relates back to the classification system we started using in 1982. Back then, a lymph node read by a pathologist was SLL, but a bone marrow biopsy (or peripheral blood) would be read as CLL. In 1994, SLL and CLL were combined into one diagnosis, CLL/SLL."
There's more about the history of CLL/SLL and the WHO classification of haematological neoplasms in this post.
The more I think about the conversation yesterday I think the young doctor (who is likely early in her fellowship at NIH) may have never seen a treatment naive patient with a normal ALC. That's what stands out to me. She mentioned my current 1.91 ALC then said CLL requires 5.0. Then she looked back at my prior labs and saw my numbers all below 5.0 except at diagnosis when I was at 5.68.....but that was coincident with my prostate surgery. My ALC has dropped each blood draw since diagnosis in Feb.When she said, you have lymphoma not leukemia my response should have been, "for now". As Newdawn points out...the presentation can change.
My diagnosing specialist (haematologist) correctly simply said I had stage 4 CLL/SLL. There were enough clonal cells in my blood for flow cytometry to identify the classic CD markers for CLL/SLL, but it then took about 2 years for my presentation to change to "CLL", as my lymphocyte count gradually increased to be consistently over 5.0. This was an arbitrarily decided threshold by CLL researchers Kipps, Rai and Hamblin over a meal. Some documents specify a clonal count exceeding 5.0. Importantly, only some cases of "SLL" progress to "CLL"; it's not a given. Irrespective, you have a clonal population of B-cells that are affecting your health in well understood ways, but it could be many years before you may need treatment.
Thanks Neil. I do have it my blood because my initial diagnosis was via PB FLOW. Then they did a LN biopsy and found the same thing. Thankfully they only found small cell in the lymph nodes and stated no large cell or aggressive cells were seen.
I´m going to NIH next week. Am really looking forward to it. What´s not to like about getting a total work up, followed by a session with some of the best experts in the field? I don't expect any surprises. Though I would like to become more proficient in 'reading' my lymph nodes. Will report after my visit. Thanks to all those who contribute here - it helps having a community that helps keep us updated.
I have been in the NIH CLL Natural History Study since 2019, although I suspended visits during COVID until August 2023, when I returned. I agree that the opinions of those experts are valuable and I like knowing that my data is contributing to the prognostic advances with molecular testing. I am w&w for 10 years, although I currently have AIHA under treatment a second time, which is a relapse from 3 years ago.
Content on HealthUnlocked does not replace the relationship between you and doctors or other healthcare professionals nor the advice you receive from them.
Never delay seeking advice or dialling emergency services because of something that you have read on HealthUnlocked.
They became a common disease with the 1994 World Health Organization classification update of haematolymphoid tumours and lymphoid neoplasms.
Mutated and unmutated cll for dummies
Diagnosed with Richters
New Diagnosis :what does all this blood work mean? 
Some answers back from doctor. 
