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Treatment for NLPHL in abdomen only

I am searching for folks who have been or are being currently treated for nodular lymphocyte predominant Hodgkins lymphoma.  Because this exists only in my abdomen (mesentery lining around small intestines), doctors do not recommend radiation therapy (because of the risk of secondary cancers).  One doctor recommends R-CHOP, but 2 other lymphoma teams recommend treatment with only Rituxin for now.  The hope is to shrink the enlarged lymph nodes.  I also have a localized schleroderma called Morphea (an autoimmune disease).  Doctors are concerned that the chemotherapy drugs (CHO) will had an adverse effect on the immune system because of the Morphea. 

Has anyone with NLPHL been treated with only the Rituxin and had success? 

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Comments

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    NLPHL

    Welcome to these Boards, Mary.

    About five of us here in the last few years were patients with your strain of HL (Hodgkins's Lymphoma) known as NLPHL. It is the rarest of the five types of HL. Your case must be very early, since the doctors are considering the Rituxan-only approach. I have not heard of that before, but it points out one thing that is unique about NLPHL:  It is the ONLY form of HL that Rituxan is useful against.  That is, virtually all lymphomas that receive Rituxan are Non-Hodgkin's forms ("NHL"). This is because Rituxan kills a cancer cell known as CD-20, and NLPHL is the only form of HL that stains for CD-20.   NLPHL in fact is somewhat of a hybrid form of lymphoma, and for some years in the past oncologists classified NLPHL as NHL, but the worldwide standard today is to consider it an anomoly of HL.

    A second curiosity you mentioned is that your oncologist was considering R-CHOP. While I have heard of that a few times in the past, almost everywhere today R-ABVD is first choice agains NLPHL.  CHOP is considered a NHL combination, and ABVD an HL combination, but there are exceptions, and this again shows how close NLPHL is to being a NHL form.  Finally, your doctors "did not recommend radiation."   Radiation is seldom given to advanced stage disease; when lymphoma patients get radiation it is very nearly always either for Stage I or II.  Or, the patient with advanced disease will occasionally have a huge mass that needs debulking, and it will be radiated. But otherwise, radiation is little used against any form of HL, so it is not surprising that they recommend not using it, at least at present.  I am unfamiliar with Morphea, but it is an example of your doctors wisely trying to do a work-around between diseases.

    My own NLPHL was very advanced Stage III, from the collar to the pelvic region, and across both sides.

    NLPHL is a very indolent, or non-aggressive lymphoma.  Depending on your age and other factors, some doctors think it best to minimize the harshness of chemo for as long as possible.  Since Rituxan is usually effective against CD-20, it seems very reasonable to hold back NLPHL with that drug; it may do so for a long, or even a very long, time.  My understainding is that Rituxan by itself is not CURATIVE against NLPHL, whereas R-ABVD is regarded as clinically curative, meaning that it will eliminate all traces of the disease for years, usually for forever (relapse following ABVD for NLPHL is 15%, meaning that 85% of NLPHL patients who get ABVD never see the cancer again in their lifetimes).

    CHOP and ABVD are both harsh.  Rituxan is not a classical chemo, but rather a form of antibody. It too can be unpleasant, but most doctors and patients agree that it is pretty mild compared to CHOP. It will not cause the patient to loose their hair, or most of the other tough side-effects that traditional chemos cause.

    I owuld ask the doctors what they think about a few cycles of R-ABVD, toward curative elimination now.  They might have compelling reasons to NOT do that. All of us writers here are not medical professionals; we write of what we have experienced, read, and studied.  I tell people that "I have no medical training; I have just had all medical problems !"  

    My summation of all of the above is that Rituxan-only, given the very early stages of your NLPHL, its indolent nature, and your immune system issues, might make Rituxan-only at this time a very sound choice.

    I hope you ask more questions and enjoy the fine people here,

    max

  • Mary from NJ
    Mary from NJ Member Posts: 60

    NLPHL

    Welcome to these Boards, Mary.

    About five of us here in the last few years were patients with your strain of HL (Hodgkins's Lymphoma) known as NLPHL. It is the rarest of the five types of HL. Your case must be very early, since the doctors are considering the Rituxan-only approach. I have not heard of that before, but it points out one thing that is unique about NLPHL:  It is the ONLY form of HL that Rituxan is useful against.  That is, virtually all lymphomas that receive Rituxan are Non-Hodgkin's forms ("NHL"). This is because Rituxan kills a cancer cell known as CD-20, and NLPHL is the only form of HL that stains for CD-20.   NLPHL in fact is somewhat of a hybrid form of lymphoma, and for some years in the past oncologists classified NLPHL as NHL, but the worldwide standard today is to consider it an anomoly of HL.

    A second curiosity you mentioned is that your oncologist was considering R-CHOP. While I have heard of that a few times in the past, almost everywhere today R-ABVD is first choice agains NLPHL.  CHOP is considered a NHL combination, and ABVD an HL combination, but there are exceptions, and this again shows how close NLPHL is to being a NHL form.  Finally, your doctors "did not recommend radiation."   Radiation is seldom given to advanced stage disease; when lymphoma patients get radiation it is very nearly always either for Stage I or II.  Or, the patient with advanced disease will occasionally have a huge mass that needs debulking, and it will be radiated. But otherwise, radiation is little used against any form of HL, so it is not surprising that they recommend not using it, at least at present.  I am unfamiliar with Morphea, but it is an example of your doctors wisely trying to do a work-around between diseases.

    My own NLPHL was very advanced Stage III, from the collar to the pelvic region, and across both sides.

    NLPHL is a very indolent, or non-aggressive lymphoma.  Depending on your age and other factors, some doctors think it best to minimize the harshness of chemo for as long as possible.  Since Rituxan is usually effective against CD-20, it seems very reasonable to hold back NLPHL with that drug; it may do so for a long, or even a very long, time.  My understainding is that Rituxan by itself is not CURATIVE against NLPHL, whereas R-ABVD is regarded as clinically curative, meaning that it will eliminate all traces of the disease for years, usually for forever (relapse following ABVD for NLPHL is 15%, meaning that 85% of NLPHL patients who get ABVD never see the cancer again in their lifetimes).

    CHOP and ABVD are both harsh.  Rituxan is not a classical chemo, but rather a form of antibody. It too can be unpleasant, but most doctors and patients agree that it is pretty mild compared to CHOP. It will not cause the patient to loose their hair, or most of the other tough side-effects that traditional chemos cause.

    I owuld ask the doctors what they think about a few cycles of R-ABVD, toward curative elimination now.  They might have compelling reasons to NOT do that. All of us writers here are not medical professionals; we write of what we have experienced, read, and studied.  I tell people that "I have no medical training; I have just had all medical problems !"  

    My summation of all of the above is that Rituxan-only, given the very early stages of your NLPHL, its indolent nature, and your immune system issues, might make Rituxan-only at this time a very sound choice.

    I hope you ask more questions and enjoy the fine people here,

    max

    Hi Max,

    Hi Max,

    Thank you so much for taking the time to provide so much information. Although I have a wonderful family, I can already see that this network is going to be my best support. I have done a great deal of reading, but none of the doctors suggested R-ABVD treatment.  I am going to do more research on this.  I was told that chemotherapy drugs would be avoided for as long as possible because of the morphea. Thank you again for replying and for the great information! Mary

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    Possibilities

    This site has descriptions of any and all chemo drugs.  Whatever you are perscribed, it describes.  Note that the menu can be for individual drugs, or for the Acronyms, that are so common in chemotherapy.

    http://chemocare.com/chemotherapy/drug-info/Rituxan.aspx

    http://chemocare.com/chemotherapy/acronyms/abvd.aspx

    max

  • Mary from NJ
    Mary from NJ Member Posts: 60
    Great info

    Hi Max ....Thank you for sending the links to chemocare.com.  I have read through the information and have added more questions to my list for the oncologist when I return next week. When you received the ABVD treatment, did you also have Rituxan?  Based on what you have read in the discussion boards, does it seem that most people have received the R-ABVD for NLPHL?  Thanks!

    Mary

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    edited May 2016 #6

    Great info

    Hi Max ....Thank you for sending the links to chemocare.com.  I have read through the information and have added more questions to my list for the oncologist when I return next week. When you received the ABVD treatment, did you also have Rituxan?  Based on what you have read in the discussion boards, does it seem that most people have received the R-ABVD for NLPHL?  Thanks!

    Mary

    Late

    I am sorry that I did not see your question for some time, Mary.

    I would say that about 50% of the NLPHL patients here got Rituxan with their abvd, and about half did not. I DID receive Rituxan.

    In my reading and experience, I would extimate that around 80% or more of the patients and Journal articles describe NLPHL being treated with abvd; chop is discussed much less often. Only one or two NLPHL patients here over the years have said that they got CHOP, that I can recall. This is all from my memory, and not a scientific determination.

    To repeat: None of us here is a doctor, and your oncologist is the one person best able to determine what treatments are best for you, not me or anyone at this Board. But I would ash why he chose what he did.

    I have attempted to learn why some NLPHL get Rituxan and some don't but do not have a totally clear answer. Sadly, at least part of the answer is insurance-related:  the diagnosis of NLPHL in some policies apparantly does not readily pay for Rituxan, but I do not believe that that is the only factor.

    Rituxan kills the CD-20 cancer cell.  Of the five major forms of Hodgkin's, NLPHL is the ONLY one that has the CD-20 cell, and NLPHL is so rare that in general, when insurance companies and doctors think of a Hodgkin's patient, they are not thinking Rituxan.  NLPHL constitutes arond 5% of all new HL cases, and around 1% or less of all new lymphoma cases in the US annually.  There are few strong or clear treatment suggestions for NLPHL, because it has been much less studied than the more common strains.  In fact NLPHL has a times been classified as a rare Non-Hodgkin's form, but for many years has been put back in the HL camp in most nations. That is, today NLPHL is regarded as a hybrid HL strain by almost everyone.   The fact that NLPHL was regarded as a form of NHL may account for why some medical centers even treat the disease with the most common NHL combination, "CHOP."   All of these things play some role in NLPHL patients getting Rituxan, or not.

    Ask you oncologist directly if Rituxan would be good for you. He will tell you.  I have suspected that some of us show lots of CD-20 cells, while othes do not, but I have no evidence of that, and may be incorrect.  That information would come from your biopsy report.

    Given the option of getting Rituxan or not, I would request it myself, but I always wanted to maximize my treatments, rather than minimize them.  In "a goody bag of abvd," adding Rituxan is usually not too bad, except that it is administered pretty slowly, and lengthens the infusion day. My infusion day lasted eight hours: I started at 8:00 AM, and left at around 4:00 PM. Take a book with you!  Rituxan can cause muscle cramps and flulike feelings, as well as respritory symptoms (runny nose, cough) but two of the other abvd drugs do that anyway.  By itself, Rituxan does not cause hair loss or nausea, but you will have no hair after a few abvd treatments; I even lost all of my eyebrows and eyelashes, and even my nose hairs.  If I had been a dog during chemo, I would have been the breed known as the Mexican Hairless !

    I am interested to hear what your oncologist says about this, since it is a fairly common question among NLPHL patients.

    max

  • Jeff148
    Jeff148 Member Posts: 184
    That is exactly what I had.

    If you click on my picture it will take you to additional info and my blog address.

    Jeff

  • Jules21
    Jules21 Member Posts: 1
    edited March 2017 #8
    newly diagnosed as the same

    Stage I maybe II NLPHL in abdomen, found during appenditicitis 3 wks ago, 3cm node removed so already has excision biopsy during an aggressive surgery, diagnosed last week following two different locations running pathology, no Rituxan yet (chemo and radiation not recommended at this time for me either), recommends repeat PET in 4mo (need a few months post-op so I don't get false positive PET due to current surgery trauma/inflammation).  What do you think of this wait and see treatment plan?  I am also getting a colonoscopy (another area lit up transverse during PET prior to surgery).

  • Jeff148
    Jeff148 Member Posts: 184
    edited March 2017 #9
    Wait and see

    I know that NLPHL has a slow growth rate, but I wanted treatment right away. I would get a second opinion if you are having doubts.

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    edited March 2017 #10
    Jules21 said:

    newly diagnosed as the same

    Stage I maybe II NLPHL in abdomen, found during appenditicitis 3 wks ago, 3cm node removed so already has excision biopsy during an aggressive surgery, diagnosed last week following two different locations running pathology, no Rituxan yet (chemo and radiation not recommended at this time for me either), recommends repeat PET in 4mo (need a few months post-op so I don't get false positive PET due to current surgery trauma/inflammation).  What do you think of this wait and see treatment plan?  I am also getting a colonoscopy (another area lit up transverse during PET prior to surgery).

    Just me

    "Active Survellance" is very common with Prostate Cancer (I learned the hard way) and a few others.

    I would never agree to it for any form of cancer -- ever. Just me.

    max

  • Hockeymomof2
    Hockeymomof2 Member Posts: 4
    Newbie - suspected NLPHL

    Hello,

    I have just come across this site as I seach the internet for infomation on NLPHL.  I have had a CT and Core Needle biopsy where the pathology report came back as highly probable for NLPHL but inconclusive as the there was nt enough tissue sample.  I am having a surgical biopsy hopefully mid April to get something difinative.  Just from the little bit of reading I have done it appears that this is a rare HL type that reponds relatively well to treatment.  I should say I am a 47 year old female, with few symptioms.  A radiologist found a 4.5 cm mass on my mesentry during an ultrasound for something else.  A follow up CT confimed what the US showed.  Unfortunatly becasue of the location of the mass (base of the central mesentry anterior to the aorta) they tried to get a core needle biopsy hoping to avoid surgery, unfortunatly it did not net enough tissue so next step is surgical biopsy for confirmation.  

    I will read back on older posts to see what others experience has been.  Thanks for sharing your stories!

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **

    Newbie - suspected NLPHL

    Hello,

    I have just come across this site as I seach the internet for infomation on NLPHL.  I have had a CT and Core Needle biopsy where the pathology report came back as highly probable for NLPHL but inconclusive as the there was nt enough tissue sample.  I am having a surgical biopsy hopefully mid April to get something difinative.  Just from the little bit of reading I have done it appears that this is a rare HL type that reponds relatively well to treatment.  I should say I am a 47 year old female, with few symptioms.  A radiologist found a 4.5 cm mass on my mesentry during an ultrasound for something else.  A follow up CT confimed what the US showed.  Unfortunatly becasue of the location of the mass (base of the central mesentry anterior to the aorta) they tried to get a core needle biopsy hoping to avoid surgery, unfortunatly it did not net enough tissue so next step is surgical biopsy for confirmation.  

    I will read back on older posts to see what others experience has been.  Thanks for sharing your stories!

    Welcome

    Welcome Hockeymom,

    Yes, a definitive biopsy result is what you need. Treatment cannot begin on a "maybe"  -- it could do more harm than good.

    If you have only the one, or a few nodes, that's good, although late-stage indolent Lymphomas are usually no harder to cure than early-stage disease.  If it is confirmed as NLPHL, expect to begin R-ABVD, which is almost universally the choice for  first-line NPLHL treatment, although Stanford and perhaps a few other places commonly use R-CHOP.  If it is indeed very early, they might want to use a little radiation and a couple of cycles.  If it is later stage, radiation is usually avoided and the number of infusions is increased.

    I hope yo ushare what the biopsy discovers,

    max

     

  • Hockeymomof2
    Hockeymomof2 Member Posts: 4

    Welcome

    Welcome Hockeymom,

    Yes, a definitive biopsy result is what you need. Treatment cannot begin on a "maybe"  -- it could do more harm than good.

    If you have only the one, or a few nodes, that's good, although late-stage indolent Lymphomas are usually no harder to cure than early-stage disease.  If it is confirmed as NLPHL, expect to begin R-ABVD, which is almost universally the choice for  first-line NPLHL treatment, although Stanford and perhaps a few other places commonly use R-CHOP.  If it is indeed very early, they might want to use a little radiation and a couple of cycles.  If it is later stage, radiation is usually avoided and the number of infusions is increased.

    I hope yo ushare what the biopsy discovers,

    max

     

    Thanks Max, I will certainly

    Thanks Max, I will certainly share once I have the final biopsy results.  They tell me there are multiple masses but I am hopeful that its early and therefore spread is limited.  Interestingly I had a ultra sound in July of 2016 and there was no mass and in Feb 2017 there was several with the larget being 4.5 cms - I am hopeful that that is a good sign that is has only been there for a short time.  

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    edited April 2017 #14

    Thanks Max, I will certainly

    Thanks Max, I will certainly share once I have the final biopsy results.  They tell me there are multiple masses but I am hopeful that its early and therefore spread is limited.  Interestingly I had a ultra sound in July of 2016 and there was no mass and in Feb 2017 there was several with the larget being 4.5 cms - I am hopeful that that is a good sign that is has only been there for a short time.  

    Odd

    Actually that is a little odd for NLPHL, which tends to grow very slowly.   To have got that big since July may suggest that it is a different strain, NOT NLPHL.  NLPHL is commonly misidentified.  It is fundamental and critical that an excise (surgically removed) biopsy precisely determines what you have.  What treats one strain of NL or NHL is ineffective on others.

    I understand that Jeff148 above has a blog regarding his NLPHL experience that you may want to start reading.  It is linked on his "About Me" section here at CSN.  Go to Jeff's About Me, and on the far right, in Blue, is his blog link.

    And know this: widespread, even advanced stage 4 NLPHL is ordinarily put into CR without too much fuss (within 6 months chemo or thereabout).  I had it from my neck to pelvic region, and from axillary (armpit) to axillary.  It was wrapped around my escophagus, crowding my heart cavity, and elsewhere. My excise node in the (L) axillary was golfball-sized.   It is easily beaten, nearly always.

    Please do write and continue updating,

    max

  • Galllessgal
    Galllessgal Member Posts: 1
    edited June 2018 #15
    Just diagnosed with NLPHL

    Hello!

    First off, I can't tell you how amazing it has been to find a board focused not only on those diagnosed with NLPHL, but those who have had lymph node enlargement in their mesentery. From much of the reading I've done and from what my oncologist has told me, it's really not as common for this disease to present in the mesentery, so it has been reassuring to know that I'm not the only one with this uncommon presentation.

    A little background: I've been dealing with illness and inconclusive symptoms for a few years now, the main two being extreme bouts of fatigue, and bouts of diarrhea that have become more frequent over the past three years. I also have had a slew of colds and seemed to be able to get sick quite easily, or at least easier than in the past. I only really started to feel incredibly sick in January of this year, with severe abdominal pain, nausea, lightheadedness, more fatigue, and more intense bouts of diarrhea paired with bouts of constipation. I do have a history of GI issues including gallstones and resulting pancreatitis (gallbladder removed in 2012), stomach ulcers, and GERD, so my GP and GI put me on a massive dose of PPI's and told me to hold out for a few weeks while they started to work.

    Lo and behold I didn't feel any better after a few months, so I went back to my GP in tears saying that I just don't feel right and I really feel like something more must be going on. She ordered a CT and in April I was diagnosed with a mesenteric mass; a PET CT one week later showed inflammation or disease in the 3.3cm mass and was told that it looked like I had a carcinoid tumor (especially based upon my symptoms). The PET also showed up to a dozen other enlarged masses, but they were not exhibiting disease or inflammation in the scan.

    After consulting with a specialist in neuroendochrine tumors and having a specialized gallium dotatate PET scan in early May, I came back negative for that type of tumor and was back to square one. Mind you that at this point, none of the oncologists or other specialists that I had consulted with had thought this could be Lymphoma. I'd already felt nuts because these symptoms are so common from many other, less scary disorders; after being told I didn't have a carcinoid tumor I started to wonder if this was all in my head.

    My surgeon convinced me that the only way to really know what this mass was is through surgical removal, so on 5/17 I had laprascopic surgery to remove the mass - a week later on 5/22 my surgeon called saying that it looked like I had a rare version of lymphoma. Two days later I saw a hemotologist oncologist aat UCLA who finally diagnosed me with NLPHL. While the blow of a cancer diagnosis was huge, for the first time in months (or years, really), I finally felt like I wasn't crazy, that the fatigue wasn't just me being lazy or not taking care of myself. It's been freeing in a way.

    I will be starting a BR regimen (Bendamustine and Retuximab) in the next week or two - four three week cycles followed by a PET scan to look for disease, then up to two more cycles if needed. Bendamustine doesn't have a clear record of causing or not causing damage to the ovaries, but to be safe we are highly considering egg retrieval prior to starting chemo since it does contain alkylating agents. My oncologist, who I trust implicitly, said that in recent studies BR has an over 90% success rate (100% in some studies) at treating NLPHL, and is much better tolerated than R-Chop or R-AVBD by most patients, with far fewer side effects. What really hit home was when he told me that I'd likely feel better after the first dose of this protocol than I have been feeling over the past few years.

    I'm wondering, did no one else's doctors discuss BR protocol with them as opposed to R-CHOP and R-AVBD? It was such a straighforward decision for my doctor, but the factors of age (I'm 33), the fact that I want to have children, and disease stage may have been an influence in his decision.

    As far as radiation, it was a moot point in the discussion of my care since my largest mass was in my mesentery and it has now been removed. It also sounds like targeted radiation is pretty impossible for mesenteric masses because of their location. Has anyone heard differently?

    Has anyone else had any overt symptoms related to their NLPHL? Sounds like many people just find a mass and go from there. Anyone else with fatigue?

    Hoping everyone is doing well with treatment and I so appreciate any insight compared to your own journeys!

    Sonja

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    Welcome

    Welcome to you Sonja. 

    I applaud that you are apparantly such a quick study, and learning about our shared disease.  As you can see from this thread, there are a few members here with NLPHL, which constitutes less than 1% of all newly-diagnosed lymphomas annually.  It is 5% of new HLs, but HL in turn is much, much less common than NHL.  To my knowledge I am the only NLPHL guy still actively writing, but may be incorrect in that.  I would say that about one new NLPHL person a year begins active participation.  While < 1% may seem like a very small number, NLPHL nonetheless remains a lot more common than some of the extremely rare lymphomas, like the T-cell and CSN (central nervous system) varieties.

    I do not have time to reread what I wrote above on this thread (or other threads), so bear with any redundancy.

    NLPHL is very indolent, and ordinarily easily put into full remission, although lifetime relapse potential is around 15%.  I can recall a few here who had refractory disease, or disease that would not go to NED (no evidence of disease, or complete remission).

    ABVD is the most common treatment for NLPHL in ternationally, although a few Cancer Centers sometimes use CHOP, a mostly NHL regimin.   I do not recall ever hearing of anyone before using B&R, although B&R is growing in popularity, and new sets tend to get experimented with on other disease strains over time.  NLPHL is actually the ONLY HL that EVER receives Rituxan anyway, because no other HL presents the CD-20 cell, which is what Rituxan kills exclusively.  This shows of course that NLPHL is an anomolous form, and more like NHL than any other form of HL.  In various countries over time NLPHL has even been classed as a NHL, but this is rare today.

    Presentation in the gut is rare; axillary is most common, followed by lower neck.  Presentation is usually early-stage, but we have had some present here as advanced Stage IV. I presented as an advanced Stage III, with grossly large nodes in the heart cavity, wrapped around the esophagus, around the superior vena cava, in both axillaries, all over the spleen, and on both lungs. Yet six months of R-ABVD cleared everything, with full remission now since 2010.  I did later develop wholly unrelated prostate cancer however.

    Radiation treatment of any Lymphoma past stage I is very rare, since it is a systemic disease. And adding radiation to many chemos confers a slight increase in the chance of later developing a leukemia.  Leukemia and Lymphoma are sister WBC diseases.

    It will be interesting to see how effective B&R is, but I suspect it will work well. Best of luck and please become a regular throughout this journey,

    max

     

  • Bill_NC
    Bill_NC Member Posts: 133
    It's been a while.

    Hi all, Hi Max & Jeff148. Although I am not an active contributor. I still visits the site most often and read the latest. The reason I am writing today as I received the bad news from My last CT scan. it's been exactly 5 years since my last chemo infusion for LNPHL. and this last scan shows couple node enlarged in the left external iliac adenopathy (abdomen left side) worrisome for recurrent disease. One of these nodes is measuring 1.3 x 02.1 cm in size. A second node is measuring 1.5 x 1.9 cm in size. and I am waiting to do the PET scan and ONC explained to me it's very hard to get to the nodes with surgery as it's hidden deep in small intestines. I am really worry what the next approach will be. if it light up in PET scan, how it will be diagnosed and treat it. will he go with radiation since it's unknown. It's all deja vo again. 

  • Max Former Hodgkins Stage 3
    Max Former Hodgkins Stage 3 Member Posts: 3,688 **
    Fifteen Percenter.....

    Hey Bill.

    I'm very disappointed to hear of your apparant relapse, something that occurs among around 15% of all NLPHL patients.  I have my own annual oncology check this week. I'm in a lifetime followship program run by my cancer center.  I had a CBC a few months ago after an ER visit due to extreme blood pressure, and it was all normal, but I have been having extreme itching for a few months now also, so checking is well advised.  I have not had any other symptoms.

    I have read some articles this year about new drugs used against relapsed NLPHL, some of which show substantial promise. I will look for those.  You must have a biopsy, since NLPHL often (not usually, but often) does not return as NLPHL, but rather as different strains, often Large-B NHLs.  To treat properly, exactly what the node is must be established.  But an irony of this is that Large-B is easier to permanently eradicate than indolent HL anyway.

    Radiation therapy (RT) as a treatment is highly unlikely.  I've never read of RT as a salvage therapy for NLPHL, but that of course does not mean that it has never been tried; perhaps some reader here will share.   Until a few years ago, stem cell transplantation (SCT) was the most common second-line approach.

    Please share all developments, since ours is a rare disease with few established protocols,

    max

  • PBL
    PBL Member Posts: 328 **
    Hello Bill,

    Hello Bill,

    I am sorry to read about your latest news. Although yours is not the same brand of lymphoma as mine, I would assume, based on my personal experience with absolutely no nodes available for biopsy (Primary Bone Lymphoma...) that the PET will help doctors see if there are any hot spots aside from your two swollen iliac nodes, for instance, in your bone marrow. The orthopaedic surgeon would then go for a chunk of bone to nail diagnosis.

    Or course, there may still be a chance that this is just about two unhappy nodes reacting to some passing bug - and then, it will just have been a little scare...

    Do keep us posted.

    Wishing for the best,

    PBL

  • Zentao
    Zentao Member Posts: 5
    edited July 2018 #20
    Bill_NC said:

    It's been a while.

    Hi all, Hi Max & Jeff148. Although I am not an active contributor. I still visits the site most often and read the latest. The reason I am writing today as I received the bad news from My last CT scan. it's been exactly 5 years since my last chemo infusion for LNPHL. and this last scan shows couple node enlarged in the left external iliac adenopathy (abdomen left side) worrisome for recurrent disease. One of these nodes is measuring 1.3 x 02.1 cm in size. A second node is measuring 1.5 x 1.9 cm in size. and I am waiting to do the PET scan and ONC explained to me it's very hard to get to the nodes with surgery as it's hidden deep in small intestines. I am really worry what the next approach will be. if it light up in PET scan, how it will be diagnosed and treat it. will he go with radiation since it's unknown. It's all deja vo again. 

    Hi Bill,

    Hi Bill,

    NLPHL relapser speaking here. Had my first bout in the year 2002 - and now gone through my second bout (diagnosed early 2018). Have done now 4 rounds of R-CHOP and have been told I am in complete remission (2 more rounds to go). 

    Happy to answer any specific questions you might have. In my case many of my 2nd round nodes (had 5) were in a similar location to yours. For the actual diagnosis, we tried 2 Core Needle Biopsies but these proved inconclusive (I knew going this was a possibility in particular if dealing with NLPHL). Ended up having a laparoscopy.

    Not sure what kind of chemo regimen you've had in the past - but R-CHOP has proven to be manageable/tolerable to me, although of course it's not a walk in the park. All in all, the biggest side effect has been fatigue. 

    I've opened up a separate thread as well, you'll find a bit more of information on my case there.

  • Zentao
    Zentao Member Posts: 5
    edited July 2018 #21

    Just diagnosed with NLPHL

    Hello!

    First off, I can't tell you how amazing it has been to find a board focused not only on those diagnosed with NLPHL, but those who have had lymph node enlargement in their mesentery. From much of the reading I've done and from what my oncologist has told me, it's really not as common for this disease to present in the mesentery, so it has been reassuring to know that I'm not the only one with this uncommon presentation.

    A little background: I've been dealing with illness and inconclusive symptoms for a few years now, the main two being extreme bouts of fatigue, and bouts of diarrhea that have become more frequent over the past three years. I also have had a slew of colds and seemed to be able to get sick quite easily, or at least easier than in the past. I only really started to feel incredibly sick in January of this year, with severe abdominal pain, nausea, lightheadedness, more fatigue, and more intense bouts of diarrhea paired with bouts of constipation. I do have a history of GI issues including gallstones and resulting pancreatitis (gallbladder removed in 2012), stomach ulcers, and GERD, so my GP and GI put me on a massive dose of PPI's and told me to hold out for a few weeks while they started to work.

    Lo and behold I didn't feel any better after a few months, so I went back to my GP in tears saying that I just don't feel right and I really feel like something more must be going on. She ordered a CT and in April I was diagnosed with a mesenteric mass; a PET CT one week later showed inflammation or disease in the 3.3cm mass and was told that it looked like I had a carcinoid tumor (especially based upon my symptoms). The PET also showed up to a dozen other enlarged masses, but they were not exhibiting disease or inflammation in the scan.

    After consulting with a specialist in neuroendochrine tumors and having a specialized gallium dotatate PET scan in early May, I came back negative for that type of tumor and was back to square one. Mind you that at this point, none of the oncologists or other specialists that I had consulted with had thought this could be Lymphoma. I'd already felt nuts because these symptoms are so common from many other, less scary disorders; after being told I didn't have a carcinoid tumor I started to wonder if this was all in my head.

    My surgeon convinced me that the only way to really know what this mass was is through surgical removal, so on 5/17 I had laprascopic surgery to remove the mass - a week later on 5/22 my surgeon called saying that it looked like I had a rare version of lymphoma. Two days later I saw a hemotologist oncologist aat UCLA who finally diagnosed me with NLPHL. While the blow of a cancer diagnosis was huge, for the first time in months (or years, really), I finally felt like I wasn't crazy, that the fatigue wasn't just me being lazy or not taking care of myself. It's been freeing in a way.

    I will be starting a BR regimen (Bendamustine and Retuximab) in the next week or two - four three week cycles followed by a PET scan to look for disease, then up to two more cycles if needed. Bendamustine doesn't have a clear record of causing or not causing damage to the ovaries, but to be safe we are highly considering egg retrieval prior to starting chemo since it does contain alkylating agents. My oncologist, who I trust implicitly, said that in recent studies BR has an over 90% success rate (100% in some studies) at treating NLPHL, and is much better tolerated than R-Chop or R-AVBD by most patients, with far fewer side effects. What really hit home was when he told me that I'd likely feel better after the first dose of this protocol than I have been feeling over the past few years.

    I'm wondering, did no one else's doctors discuss BR protocol with them as opposed to R-CHOP and R-AVBD? It was such a straighforward decision for my doctor, but the factors of age (I'm 33), the fact that I want to have children, and disease stage may have been an influence in his decision.

    As far as radiation, it was a moot point in the discussion of my care since my largest mass was in my mesentery and it has now been removed. It also sounds like targeted radiation is pretty impossible for mesenteric masses because of their location. Has anyone heard differently?

    Has anyone else had any overt symptoms related to their NLPHL? Sounds like many people just find a mass and go from there. Anyone else with fatigue?

    Hoping everyone is doing well with treatment and I so appreciate any insight compared to your own journeys!

    Sonja

    Hi Sonja,

    Hi Sonja,

    NLPHL relapser here. Currently undergoing R_CHOP and have been confirmed last week I am in complete remission (!!).

    Had NLPHL 2 times. Second time was a IIA, located in the lower abdomen. No specific symptoms other than some mild discomfort which for some reason triggered me to get really anxious about it. The actual nodes were small and 3 doctors believe their finding was incidental but i believe otherwise.

    Regarding radiation, this is very specific to each case. But in mine although it was considered as an option (my first treatment was radiation only in the neck and responded very well) it was dropped because of location as you mentioned, coupled with the fact that I was relapsing after 15 years and therefore my hematologist leaned on a more systemic approach.

    Happy to answer any specific questions you might have - just let me know.