Just Diagnosed Advanced Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL)

Jon_Paul said:

NLPHL

Update

Welcome, Jon-Paul.

Many first-timers provide little detail; such was not your case. But more specifics are better than fewer.

You have found one of the more active NLPHL threads -- a great place to jump onboard.

I too had NLPHL (as following this tread has shown you), advanced stage 3, from the collar bone to the pelvic region, across axillary to axillary. Inside both lungs, wrapped around various organs.  NLPHL constitutes around 5% of all new HLs, but since HL is much less common than NHL, NLPHL is only about 1% or less of all newly-diagnosed lymphopmas annually.

It is quite indolent, or slow-growing, lacking in aggressiveness.  Because of its rarity, NLPHL lacks definitive medical recommendation for treatment, and in times past NLPHL was classified as a rare NHL, and some nations still regard it as such.  It absolutely lacts the Reedberg cells that are the hallmark of HL, and does stain for CD20, which no other form of HL presents.  Too few case studies with too few patients have left various schools of thought arguing about how to treat it, or when.  In times past early stage was sometimes treated exclusively with RT.

The best studies today recommend aganst solo RT treatment at any stage, showing much better long-term freedom from relapse with combined modality treatmetns that include chemotherapy. Or even chemotherapy alone, with no radiation. Many studies show that combining most forms of chemo with radiation statistically increase the likllihood of later morphing into a lelukemia, even if decades later. But of course, there are cases where absolutely both must be employed, to ensure immediate survival. (I am not speaking specifically of treating NLPHL with both radiation and chemo, but all chemos with radiation; there are over 200 FDA apprioved chemotherapy agents in the US currently.)

Neither I nor anyone here is a doctor or medical professional. We share layman's insight and experience.  But I absolutley would not have choosen solo RT as a form of treatment for NLPHL.  Doctors who view NLPHL primarily as aan HL ordinarily use R-ABVD, the most common first-line agent against classical HL (CHL); doctors who view NLPHL more as an oddball NHL use R-CHOP, the most common first-line combination used against most NHLs (I believe Stanford is in this latter general camp, but cannot say for certain; most of the patients here who have gone to Stanford with NLPHL reported being on R-CHOP).

NLPHL has excellent rates of CR (complete remission) or NED (no evidnece of disease), but lifetime relapse is fairly high, at about 15%. Because I was over 40 at diagnosis (53 then) and advanced stage my oncologist said I had a 60% chance of being alive in five years. Relapse of NLPHL can occur as NLPHL again, or as an aggressive NHL, or as a leukemia.   I am in CR after six years now, following massive involvement.  I have never read of a NLPHL patient here who reports relapse to date...there have been several Stage IV writers here over the years, although in my time here since 2012, I don't think I have encountered more than perhaps ten people who shared our disease.

Duke is world-class, and your RT knows what he is doing. The four of you all having cancers is indeed bizarre. May you all achieve and sustain full recovery. Yo uhave the intelligence, research, and swagger to get through this as a healthy old man.

I am going to post an article from a world-class expert regarding an overview of NLPHL here as soon as I can find it again.  Forgive the typos: permanent chemo fog, neuropathy, possible Parkinson's, and STRESS make keyboarding a challange. Grad school in Germanic history taught me the improtance of writing well, but it aint no longer an option.  And it taught me how to study fast, the old "drink from a firehose" image used in grad school. I knew absolutely nothing about lymphoma at diagnosis -- nothing.

Most people today can only Tweet 30 words or less anyway, or use emojis.  Soon society will be so dumbed-down than humans can only make grunting noises at one another.  We are devolving into what many cultural historians are referring to as a "new Barbarism": a level of crudness and lack of reason that has not been seen since prehistoric times a trend started at least by the 1950s.  I am just below Asheville in SC.  Our state motto is (from the Latin)

Whilst I breath, I hope

max

An excellent technical overview of NLPHL and its treatment history. I believe the author is a "Stanford guy."

http://www.bloodjournal.org/content/122/26/4182?sso-checked=true

nickbach said:

NLPHL story

My son was diagnosed on July 11, 2016.  It was by far the worst day of my entire existence.  He was 5 at the time - 6 now.  

Leading up to it - how we discovered it - his dentist noticed a lump in his neck area.  We had never even noticed it, but looking back at his baseball pictures, you could see it.  We were told to get it excised, no matter what it was - it was a pretty hard little mass in there.  We hoped and prayed for a "cyst" or any kind of tooth situation...but on 7/11/16, my wife got the phone call and they said "Hodgkins".  I went into a pretty dark place in my brain and lost tons of sleep.  Impossible to not think the worst, but kept telling myself to stay positive.

One of the only things I ever took comfort in was message boards like these.  Reading other people's stories and how they made it through each day gave me comfort knowing we're not alone in this thing.  That's why I'm posting here today - hopefully to help someone out - maybe the next Dad or Mom that reads this....

Initially, the diagnosis came back as "Hodgkin's"...which made me sick to my stomach.  I researched it like crazy and didn't feel any better about it.  About 2 weeks later, we received a more precise diagnosis of Stage 1 NLPHL.  I hit my knees when the Doc said "100 % cure rate" - it was the most beautiful thing my ears had ever heard.  I no longer cared about the treatment, his hair, his schooling, none of it.  All I knew was that my little boy was going to live - and that was all I cared about.  

To make a really long story short - we went through 3 rounds of CVP for chemo.  His hair never fell out.  I never even told him what was going on.  I just wanted him to keep smiling and being a little boy.  I didn't want him to think he was "different" in any sort of way.  he called his port line "his robot" and I told him he was getting a "power disk" put into his chest just like Iron Man.  He hated "getting poked" - and that was by far the hardest part of all of it - seeing  him cry...but he made it through.

Results came back 100% full remission right before Thanksgiving.  We hit the road the ext week for Disney World.  I pray every day that it's over and done with.  I also pray to the Lord that the next person in my family to get sick in any sort of serious way will be ME!!!  I think he hears my prayers, and I look forward to every day now with a very different outlook.  I count the minutes now....not the dollars.  I just want to spend as much time with my kids  as possible.  It's been a huge blessing in perspective for my wife and I - and we're all still here alive and well.  Cancer sucks - but it can be BEAT!  

#StrongLikeNate

 

Strong,

Your story regarding Nate is remarkable. Kids seldom get indolent cancers, almost always aggressive types.

Its beauty speaks for itself. Thank you.  And know that he is absolutley the youngest NLPHL report here ever, and one of the yougest lymphoma patients ever reported here as well.

max

Hey guys and girls,
my name is Stephan, I am 34 years old and I'm from Frankfurt/Germany. Since here in Germany are no good cancer forums (especially not for NLPHL) i was happy to find this one.

Short introduction to my person and the process of my cancer:

Somewhere in 2009: First time I recognized a bigger as usual lymphnode in my right groin. I didnt take it much serious.
10.12.1015: Went to a doctor and he measured it with ulrasound. It was 4 cm big in lenght. I decided to not take it out. I didnt had any B symptoms.
20.03.2017 Extraction of a 5*3 cm Lymphnode from my right groin because my doctor found in the ultrasound 2 more lymphnodes with each 2cm right beside the big one.

24.03.2017: Diagnosis NLPHL

Afterwards many visits at different oncologists and 2 CT's (Abdomen/Groin, Thorax). Unfortuntely they found 5 more "pathological" lymphnodes close to where they extracted the first one. Thorax was clean.
One of my oncs said, that it can be anyway stage IA, because the additional nodes are in two different areas, but these Areas are so close to each other that may we can consider it as one area and treat it only with IF.

Next week on the 18.04. i have my bone marrow extraction and ths is what i fear the most because of the possible result. I know that when they find there something, it will be stage IV and I'm going to have a hell of a time with Chemotherapy.

Of course I will let you all know what are the results.

Since I was reading a lot about this NLPHL i learned that IF is the common use in stage IA whereas others say chemo would be better, because the chances of relapse are smaller. Furthermore I would have to freeze my sperm before the IF because i plan to get children with my wife withinh the next year. Does anyone here maybe has any sources that can help me make a good decision on which therapy i should take?

And one more thing: I always thought i was psychological stable but since the diagnose i feel like i am getting crazy. I know the changes for cure are good but I am quite a pessimistic person adn I am **** afraid of the therapy (IF or chemo, both are a massive attack on the body). Since then I take benzodiazepine each day because otherwise i wouldn't be ablte to sleep or calm down in any way.

Hello Max,

thanks for your words, they calmed me a bit down. 
It's definitely NLPHL. I dont know how it's in the US, but here in Germany first one pathologist looks at the extracted lymphnode and afterwards another has to confirm it.
Of course, I am really "happy" to have this kind of cancer because I know it's so good to treat but I also do read about the side effects of the therapy which are for example other forms of cancer like leukemia which can come up even decades later. But then I think "hey, may in 20 years they've found a cure against all this".

Max, i read on your side here that you wrote that after this disease you will never be the same again. What exactly do you mean by this?
When this whole thing is over I dont want to be attached with it anymore and completely forget about it. I only want to have it out of my body and then never think about it anymore.

I understand what you mean.
Sure, you can also see it as something valuable since you make an experience not vereyone makes and therefore widenyour horizon. I guess it's all a matter of your personal constitution and opinion  or point of view towards the disease and life in general.
For example many people told me not to read about it in the internet because this would just make me even more crazy and feeling uncompfy, but for me it's the opposite. Maybe because the disease isnt as bad as other cancers and the more i read about that people got in full remission the more i feel safe. On the other hand many doctors tell you different way of treatment and in the end you have to choose on your own which is the best for you whereby you can only choose this if you have the needed background knowledge.

I start recognizing that the more I know about NLPHL, the less afraid I am. Anyways, there is always the problem that once you got this disease, it can come back, or even worse, another, more aggressive kind of cancer, comes back. I think this will be the thing I'll have to work with from now on.
At this point I also do have my next question: Even if i will be diagnosed in stage 1A and they offer me IF only, shall I take it? I've read that it cures the cancer but the chances that it comes back is higher and also a radio therapy can make a bigger risk for other cancers like leukemia. Maybe I should take only Chemo? I wouldnt have a problem with the side effects, they will go away, but therefore i have a bigger chance not to hear anything from cancer ever again.

And something else: Looking at the statistics for this disease, did any of you ever thought about playing the lottery? xD

It's been a long time hi all. you got this I was stage 4bes kicked its butt and still going strong. I did r-abvd for 6 months. 

Hi DJ. I had NLPHL 8 years ago. No relapses. 6 months of RCHOP. I was stage 3b. My problem started with a couple broken ribs and getting pneumonia which wouldn’t go away. Then I started getting infections. My eyes. Cut my finger and almost lost it due to 8 diff bacterial infections etc. my oncologist hadn’t ever seen this type of cancer before. Please know that you’re not alone. I don’t know where you live but maybe going to a may clinic type place would be the best bet now. If you have any questions please feel free to reach out to me. Hope you feel better.

lorraine

Anyone diagnosed with NLPHL, take note that the World Health Organization (W.H.O.) has recently re-classified NLPHL as (non-Hodgkin's) Nodular Lymphocyte Predominant B-Cell Lymphoma. It appears that US authorities have not yet made this change in diagnosis. This has been coming for some time, as Hodgkin's is a substantially different disease and treatment is different. Specifically, it will most likely now be addressed similarly to Follicular non-Hodgkin's Lymphoma. The difference will be the almost certain elimination of the Bleomycin (the "B" in ABVD) in treatment regimens. This is good as Bleomycin is known to produce long-term lung toxicity. Anytime that treatment toxicity can be reduced is a good thing. Anyone recently diagnosed might wish to ask their hematologist about this re-classification to see if the less toxic treatment is proposed.

DJ, Lorraine, and all others:

I also am a NLPHL survivor. I have written extensively regarding this strain of HL above, on this same thread. The majority of that info is still valid. Also, what Po wrote the other day is completely accurate.

I will just recap a few things: I was first diagnosed with NLPHL, advanced S-III, in 2009. I did the standard R-ABVD toward curative first-line. I went straight into complete remission (CR), which is also referred to as No Evidence of Disease (NED). NLPHL, as a highly indolent strain, almost always goes rapidly into CR, whether via R-ABVD, R-AVD, or even B &R (Bendamustine and Rituxan).

But, NLPHL is very much given to relapse; over 10 years, the rate is close to 20%. What is not yet certain is how well less toxic treatments prevent or delay relapse, despite the fact that all seem to work well initially. I relapsed in 2021, again at Stage III, bulky Disease. I am now being treated with Rituxan maintenance, and after a few month's treatments, am currently NED. This is based upon a PET, not a CT. Tumors remain present. My hematologist says that if it becomes overall refractory, stronger combinations will be needed: Usually this is B&R, but some cases suggest the need for SCT (stem cell transplant).

In most cases, NLPHL returns to CR after second-line therapy, but can recur yet again. Also, particularly after second-line therapies, NLPHL can and fairly often does morph into Diffuse Large B NHL, which nearly always requires SCT at that point.

The good news is that NLPHL routinely responds well to first, second, and even third line therapies. The bad is that it is a condition that must be watch for for life, even after rapid movement into CR with curative first line success. I won't bore you with the details, but for about a year (in 2020) my biopsies came back as an autoimmune disorder known as PTGC. PTGC is even rarer than NLPHL itself, and the two are regularly misdiagnosed, so world-class pathology is essential. I have had odd-ball autoimmune symptoms continuously since 2009: pruitus, fatigue, and a burning sensation in my nodes after drinking beer. These are all rare, but known, HL symptoms also.

As Kermit the Frog says: 'It aint easy being green.' It aint easy being us, either.

max

Hey po18guy,

Thanks for sharing about the long-term lung toxicity. I will inform my doctors next week when I see them, they are not looking at the meds in this way, but my lungs have been showing new nodules even in my thyroid on last week CT. So I will help them to expand their research with this NLPHL.

I am not giving up yet! I might have to go to TX or somewhere they are more up to date on this cancer.

Thanks again,

DJ