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

Hello, I am really happy I found this site.  After being sick for 3 1/2 years, I had a myph biopsy and was diagnosed with SUSPICIOUS for Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL).  The doctor says he contacted the pathologist and Stanford Medical to confirm.  He says I most likely have had it a long time (makes sense since it is slow moving).  I have suffered from fevers and nightsweats (whole body drenching) for more than two years which he explained are B symptoms. I had a bone marow biopsy and have another PET Scan coming up.

He mentioned combining ABVD with Radiation (he called it less agressive) but said it could come back in 10-15 years and by then there would be an easier cure.  Alternatively, he says I can be agressive with R-ABVD (I think he said this) and be rid of it.

Anyone else been through this?  

Would really like to know pros and cons.

 

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Comments

  • bros
    bros Member Posts: 4
    Bone Marrow biopsy

    with the bone marrow biopsy, if you are sensitive to pain - ask the doctor to give you a painkiller like percocet for you to take a bit before the biopsy. They can be quite... uncomfortable. It is best to dull the mind before one.

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Addendum

    Ed,

    You have what I was diagnosed with about six years ago. I was late Stage III (highly widespread disease, from my neck to pelvic region). NLPHL is very rare, constituting about 1% of new lymphoma cases per year in the US (NLPHL is 5% of new HL cases, but HL occures only about once for every three or four new cases of NHL, which comes out at approximately 1% of all lymphoma types combined).  As you noted, it is a very indolent form, quite slow-moving. When my doc told me that it grows very slowly, but that it was "all over," I asked how long I had likely had it, and he said at least a year, maybe a lot longer, which is similiar to what your doc told you. 

    I did not have "B" symptoms; in fact, I never had any symptoms, except severe fatigue. My disease was discovered by accident, during a heart scan.  First line therapies are usually effective in achieving complete remission (no ability for PET or CT to detect residual disease), but it does have a 10-15 % chance of eventual relapse, and occasionally will morph into a more aggressive strain of HL. Late stage disease, the presence of B-symptoms, and being over 60 years of age at diagnosis all lower survival statistics a little.  Former Pennsylvania Senator  Arlen Specter died of NLPHL.  He went into full remission twice, but when he relapsed the third time, it came back as an aggressive NHL, which was the end.

    But focus on the good news:  NLPHL is very treatable in virtually all cases.  I did the r-abvd option, and am clean nearly six years later.  Stage III and IV require six cycles, or twelve infusions, which takes six months of chemo. Extreme cases sometimes go eight cycles, which adds two more months.   Radiation is not very commonly used against most lymphomas, except quite minor, localized cases, or in cases of "bulky disease."  ABVD can have a lot of unpleasant side-effects, but not everyone gets these, and most diminish or go away after treatment ends.

    Rituxan is not often used against Hodgkins lymphoma (HL), but it is used with NLPHL, because NLPHL presents the CD-20 cell, which is normally just seen in NHL.

     

    I can recall two others here who had NLPHL:  "Aaron," and "Bill_NC."  We are here for you....

    max

     

    Additional:

    I typed the stuff above at work on a phone. Rereading it now, I can see that I did not explicitly answer your question. It seems the oncologist gave you an option of (abvd + rituxan) or (abvd, no rituxan, and radiation).  Part of the decision depends on staging. If you are III or IV, I would go with r-abvd, which is the normal treatment, late stage.  Rituxan is not usually considered one of the harsher chemo agents (it is technically not chemo anyway, but rather a "monoclonal antibody"). Some studies suggest that combining chemo with radiation may occasionally, years or decades later, increase the chances of getting treatment-induced leukemia. Probably very unlikely, but it is something to be aware of.

    I have never heard of an "either/or" proposition regarding radiation and Rituxan. That is, I don't think they are mutually exclusive, so why he posed the treatment options that way is worth asking him .

    I hope some of this is informative and assists you in your decision making process.

    A fairly good summation of how NLPHL is viewed by oncologists:  http://www.hindawi.com/journals/crihem/2014/427613/

    .

  • Jeff148
    Jeff148 Member Posts: 184
    Hang in there!

    Ed,

    I'm surprised that Max forgot about me. I'm a fellow NLPHLer. Max was my favorite info. provider.  I was stage one and went the route of R-ABVD. I have information on my "page" and blog. IF you click on my picture it will take you there. It's a tough battle, but winable. Stay strong and let me know if you have any questions. You can send email through this site to me. I started my chemo last year on May 1 and became NED in September.

    Jeff

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Jeff148 said:

    Hang in there!

    Ed,

    I'm surprised that Max forgot about me. I'm a fellow NLPHLer. Max was my favorite info. provider.  I was stage one and went the route of R-ABVD. I have information on my "page" and blog. IF you click on my picture it will take you there. It's a tough battle, but winable. Stay strong and let me know if you have any questions. You can send email through this site to me. I started my chemo last year on May 1 and became NED in September.

    Jeff

    Sorry !

    Jeff,

    Definitely an oversight there, Jeff !  I forgot, but neither can I remember what I had for lunch (and it is 2:30 PM local time).  I am sure it wasn't a smoothie, however.

    There was a female writer in our NLPHL club back in the day, very friendly.  I will try to recount her name as well, especially since NLPHL is infrequent among females.

    max

  • Sdgirl96
    Sdgirl96 Member Posts: 4
    I also was diagnosed with

    I also was diagnosed with this type. I am stage 1 so I only had localized radiation. I just finished treatments last week and am waiting for the next round of tests to see if it worked. Good luck on your journey!

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Sdgirl96 said:

    I also was diagnosed with

    I also was diagnosed with this type. I am stage 1 so I only had localized radiation. I just finished treatments last week and am waiting for the next round of tests to see if it worked. Good luck on your journey!

    Welcome

    Welcome to our little band of indolents, Sd !

    I hope all is clean on your pending scans,

    max

  • Katie F.
    Katie F. Member Posts: 1
    Hello all!  I am so happy to

    Hello all!  I am so happy to have found this site!  My husband (age 33) was just diagnosed with Stage IV NLPHL.  We are in the process of getting a second opinion and confirming his treatment plan.  Our first doctor is recommending 6-8 months of ABVD chemo.  In the meantime, if anyone has positive stories to share, it would mean the world!  It's hard to find a lot of information about this type of cancer at this stage.  Thank you in advance and we look forward to connecting with you all!

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Katie F. said:

    Hello all!  I am so happy to

    Hello all!  I am so happy to have found this site!  My husband (age 33) was just diagnosed with Stage IV NLPHL.  We are in the process of getting a second opinion and confirming his treatment plan.  Our first doctor is recommending 6-8 months of ABVD chemo.  In the meantime, if anyone has positive stories to share, it would mean the world!  It's hard to find a lot of information about this type of cancer at this stage.  Thank you in advance and we look forward to connecting with you all!

    Welcome

    Welcome, Katie.

    I had late-stage 3 NLPHL (very widespread).  It is usually quite treatable. It is a rare disease, constituting less than 1% of all new lymphoma cases a year (5% of new Hodgkin's cases, bu Hodgkin's is much rarer than non-Hodgkin's).  I read a report that said that all of England has fewer than 300 cases per year, average.

    What is your husband's age ?  What secondary organ did the cancer enter to render it Stage 4 ?  (Usually it is the bone marrow, but any organ is a possibility).

    R-ABVD is the usual treatment for first-line NLPHL, probably 90% of all cases. Rarely, an oncologist will use R-CHOP.  I did six months of R-ABVD, but doctors do push that up to eight months in severe cases.  Radiation and surgery are never used against late stage NLPHL, unless it it to shrink a huge node causing pain somewhere.  Due to its rarity, there is not a strict protocol for treatment, but as I said, R-ABVD is the usual choice, and seems to work very well.  Having read about it for half a decade now, ABVD would be my choice if I were beginning first-line treatment again.

    We have had about 7 or so NLPHL cases here since I joined a few years ago.  100% went in to full remission, and no one has relapsed to my knowledge. All but one used ABVD.  ABVD is ROUGH when used long-term (several months), but most side-effects go away over time.  I am almost six years clean of the disease currently.  It can relapse years later, and does so in 10-15% of all cases.  It is the cancer that Sen Arlen Spector of Pennsylvania had -- our one claim to fame !  

    Please do stay in touch and give specifics of his case.  I am sure we have valuable insights to share.

    max

  • Bill_NC
    Bill_NC Member Posts: 133
    Hello my NLPHL Buddy's

    Hello Max, Jeff. it's been a while. Missed you guys. 

    Hello and welcome ED , Sdgirl96 and Katie. 

     

    Hope all doing well. Sorry been away for long just got sucked back to the busy life. I need to slowdown and thank God for his Blessing and Mercy. Last July was my 2 years mark of NED. I was stage IIIA , I only used ABVD alone. Since my last visit to the ONC he said we are stopping the CT scan every 6 months to once a year. When I first diagnosed I never thought I'll be here witting 2 years later. 

     

    To ED and Katie husband roll your sleeves up and raise your heads up because I know you will beat that sucker.  

     

    I am sure you will see more of me here in future. All stay safe and take care.
  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Bill_NC said:

    Hello my NLPHL Buddy's

    Hello Max, Jeff. it's been a while. Missed you guys. 

    Hello and welcome ED , Sdgirl96 and Katie. 

     

    Hope all doing well. Sorry been away for long just got sucked back to the busy life. I need to slowdown and thank God for his Blessing and Mercy. Last July was my 2 years mark of NED. I was stage IIIA , I only used ABVD alone. Since my last visit to the ONC he said we are stopping the CT scan every 6 months to once a year. When I first diagnosed I never thought I'll be here witting 2 years later. 

     

    To ED and Katie husband roll your sleeves up and raise your heads up because I know you will beat that sucker.  

     

    I am sure you will see more of me here in future. All stay safe and take care.
    Delighted

    Delighted to hear you check in, Bill.  The results are wonderful.  At about three or four years NED, your oncologist will end all CTs, and just use blood panels and a physical exam to monitor you. I have not had a lymphoma CT in a few years now.  Everyone in our treatement club for NLPHL (including Aaron also) has been disease free, to my knowledge.  Matthew Butts also, who was critically ill for a time, and who had to receive his chemo as an inpatient for a month or two.  He was NED, last I heard.

    max

  • Don2088
    Don2088 Member Posts: 16

    Delighted

    Delighted to hear you check in, Bill.  The results are wonderful.  At about three or four years NED, your oncologist will end all CTs, and just use blood panels and a physical exam to monitor you. I have not had a lymphoma CT in a few years now.  Everyone in our treatement club for NLPHL (including Aaron also) has been disease free, to my knowledge.  Matthew Butts also, who was critically ill for a time, and who had to receive his chemo as an inpatient for a month or two.  He was NED, last I heard.

    max

    It has been a while since I

    It has been a while since I have posted. I was diagnosed in July with Stage 3 NLPHL. My first oncologist recommended R-ABVD. The secodn opinion oncologist from UPENN recomended R-CHOP. She said at my age (44), she would rather not give me the lung toxin in ABVD. I have had 4 R-CHOP treatments. I was scanned between 3 and 4. Everything outside my chest area was in full remission or significantly improved. There were a couple in my checst that still showed improved, but still fairly active. I have 2 more treatmetns (11/2 and 11/23). Hopefully in January you can add me to your list of NED patients.

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Don2088 said:

    It has been a while since I

    It has been a while since I have posted. I was diagnosed in July with Stage 3 NLPHL. My first oncologist recommended R-ABVD. The secodn opinion oncologist from UPENN recomended R-CHOP. She said at my age (44), she would rather not give me the lung toxin in ABVD. I have had 4 R-CHOP treatments. I was scanned between 3 and 4. Everything outside my chest area was in full remission or significantly improved. There were a couple in my checst that still showed improved, but still fairly active. I have 2 more treatmetns (11/2 and 11/23). Hopefully in January you can add me to your list of NED patients.

    Status

    Don2088,

    It sounds like your progress is tracking in a pretty routine fashion for a patient who began with widespread NLPHL.  Based on what I have read here over the years and my own, personal experience, it would have been unusual for there to have been no tumor activity remaining at this time.  Your next PET will most likely wait until at least a week or two following your 11/23 infusion.  If anything remains at that time, depending on how minute it is, a cycle or two of the R-CHOP might be added, or possibly some radiation.  This would not constitue "relapse," since first-line treatment is not yet ended.  Your oncologist will answer what he or she thinks on this issue if asked, of course.

    But we hope that all is totally and completely "clean" then.  We want you on The List !

    max

     

    .

  • Spanishlady
    Spanishlady Member Posts: 7
    Diagnosed with NLPHL

    hey all,

    finally decided it it was time to share a post. I was 48 years old when I was diagnosed with NHPHL. diagnosis did not come easily as I didn't have your typical symptoms. It was mid-July 2015 and I was experiencing a nagging unproductive cough for weeks. I went to my GP on two different occasions. The first time I was told  it was allergies, the second visit, I was told it was just a summer cold. it was the end of August and the cough was getting worse. I called my GP and demanded an X-ray. That is when my life changed as I knew it. to make a long story short, 2 weeks after the initial x-ray, I was scheduled for thoracic surgery to biopsy the 7 cm mass in my chest. I also had a 4 cm mass in my lower right lung. 

    advanced stage III NLPHL. I was given 6 cycles of APVD which began in Oct 2015. In Mid December I was ordered another PET which came back as a "complete response." March 21 was my last infusion. IN the beginming, I could deal but at halfway mark I was starting to feel more fatigued, stomach problems, and swelling of my hands and face. I can't wait to feel normal again but what is "normal?" I focused so much on getting through treatment that I had forgotten to fully grasp the emotional toll it has taken.

    this experience may have started out like a death sentence but it has made me realize that I am not in control of everything and its okay to feel vulnerable.

  • pintofplain
    pintofplain Member Posts: 1
    edited December 2016 #15
    Chiming in

    Hi all,

    Just wanted to introduce myself. I am a 38-year-old man diagnosed 11/22/16 with NLPHL, which in my case we found investigating a cluster of enlarged axillary lymph nodes. PET scan for staging found disease in my bone marrow and spleen, so my disease is stage IV. I didn't have any other symptoms aside from the lump under my arm. My oncologist at Jefferson in Philadelphia started me straight away on R-CHOP, a planned 8 rounds. I'm 8 days into my treatment, and so far, it's been ok -- and my lump has all but disappeared. Good luck to all of you who are fighting with me or in remission!

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    Welcome

    Welcome, pintofplain !

    As you can see, this thread is a detailed review of NLPHL, a rare HL.  I see you are getting CHOP, which means your doc is of the school of thought that views the strain as a variant of NHL.  Such is not uncommon, and NLPHL has gone back and forth in classification over the decades. In some European countires it is still formally classified as NHL.

    Your screen name and use of the term "straight away" makes me think you are perhaps British...?

    Almost every NLPHL is discussed above, if you want to scan through it.  Please keep writing, and feel free to email me on the CSN Email service here any time.

    max

     

  • lindary
    lindary Member Posts: 711 Member
    edited December 2016 #17
    Hello pintofplain

    Two years ago I was told I have folicular non-hodgkins lymphoma, stage IV. I had 6 cycle of R-Chop. Two things with this chemo. Wiith each treatment you will be more tired than with the previous. Second is the Prednisone. I took 100 mg daily for 5 days, starting on the first day of treatments.  It made me feel pretty good. On the 6th (first day without it) I felt ok. The 7th & 8th day had me sleeping any time I could put my head down. I found that if I took a nap on the 6th day and slept 9 - 10 hours at night the 7th & 8th days weren't quite so bad. Either way, I told my boss that the 7th & 8th days I would only wokr half a day so I could sleep the rest. 

    I found R-Chop fairly easy to deal with, except for the Prednisone. Almost felt guilt telling people I was on chemo since I wasn't having any side-effects. However for me the R-Chop didn't get it all and I had to do 3 cycles of RICE, also given every 3 weeks. For this one it was 3.5 days in the hospital getting the chemo. It hits the blood cells really hard. By the 3rd treatment I had to get blood transfusion to keep the red counts up. Came close to needing platelets too but each time my body would come back enough to avoid that transfusion. 

     

    Good luck with your treatments. 

     

  • Mabakhrts
    Mabakhrts Member Posts: 1
    edited January 2017 #18

    Chiming in

    Hi all,

    Just wanted to introduce myself. I am a 38-year-old man diagnosed 11/22/16 with NLPHL, which in my case we found investigating a cluster of enlarged axillary lymph nodes. PET scan for staging found disease in my bone marrow and spleen, so my disease is stage IV. I didn't have any other symptoms aside from the lump under my arm. My oncologist at Jefferson in Philadelphia started me straight away on R-CHOP, a planned 8 rounds. I'm 8 days into my treatment, and so far, it's been ok -- and my lump has all but disappeared. Good luck to all of you who are fighting with me or in remission!

    Nlphl

    hi! I typed this and thought it was posted but I don't see it. I was diagnosed jan2015 with nlphl stage 3b. R chop. Plus I was diagnosed with sarcoidosis st the same time. Ended up with thyroid cancer September 2015 and I am just finishing up everything with the thyroid. How are you feeling?  It's a rough regimen. 

  • Schembrak
    Schembrak Member Posts: 8
    NLPHL

    Hello, I got diagnosed with Stage 4b NLPHL with T-cell rich nodules, officially on 07/22/2016. From what my Oncologist told me....that the type of cancer I had only presented itself in only 10% of NLPHL cases. With me having 4 masses in my spleen and it being in my pelvic bones, changed from wanting to do the ABVD, to the RCHOP. He said the RCHOP is more aggressive than the ABVD. 

     

    However, I was told on December 16th that I am in FULL REMISSION!!! He has scheduled for me to get my power port out in April!

    What does NED, mean?

    Prayers to everyone!

    Kaitlyn

  • lindary
    lindary Member Posts: 711 Member
    edited January 2017 #20
    NED

    No Evidence of Disease!

    That is a great thing to hear. Congtratulations.

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,736 Member
    edited January 2017 #21
    Schembrak said:

    NLPHL

    Hello, I got diagnosed with Stage 4b NLPHL with T-cell rich nodules, officially on 07/22/2016. From what my Oncologist told me....that the type of cancer I had only presented itself in only 10% of NLPHL cases. With me having 4 masses in my spleen and it being in my pelvic bones, changed from wanting to do the ABVD, to the RCHOP. He said the RCHOP is more aggressive than the ABVD. 

     

    However, I was told on December 16th that I am in FULL REMISSION!!! He has scheduled for me to get my power port out in April!

    What does NED, mean?

    Prayers to everyone!

    Kaitlyn

    T-Cell

    Kaitlyn,

    Welcome to you. I am thankful you have written.

    NLPHL by itself is very rare, and then this T-rich substrain (as you mentioned) makes it even rarer (I am guessing, probably less than 1 in 1,000 new lymphoma cases).   It is something that the oncologist will probably want to watch for some time.  NLPHL is not a well-studied disease, and there is not a lot of research on how to best treat it, with the lucky part being that it ordinarily is easily beaten by whatever the oncologist chooses.  As your own history demonstrates, the combos of choice are almost always either R-CHOP or R-ABVD.

    I hope you recover rapidly, and choose to write in the future,

    max