Primary Bone Lymphoma, anyone?
Hi all,
I have Primary Bone Lymphoma, which is deemed a rather rare occurence of lymphoma. My variety is Follicular (indolent), which is even further off the charts (the "usual" type of PBL is DLBCL (aggressive)). Specific information is hard to come by... So I would love to find other folks with PBL, who would be willing to share their experience.
I was diagnosed with PBL a year ago, after years of unexplained bone pain and a spontaneous fracture in late June 2015.
I am being treated at a teaching hospital which has over 300 lymphoma patients a year, and "maybe one PBL a year", to quote the rheumatologist who diagnosed me.
I am currently in remission, on maintenance Rituxan (every 8 weeks for two years) after 6 R-CHOP21 (last infusion, mid-June 2016).
I have just had a most unpleasant and disturbing experience, which sparked a question to which I cannot seem to find an answer (not even the rheumatologist could tell!): in the event of a recurrence, will it be more of the same, i.e. new bone pain, fracture, etc.? What would be sure signs that it's time to make an appointment with the hematologist?
Thanks for any input.
PBL
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Maybe
PBL,
Your case is about as unusual as I have read, but our unofficial Rare Cancers person here, Po18Guy, might be able to mention some specialists or Rare Cancers Centers to you. He is expert with T-cell diseases and Stem Cell transplantation issues, among others, but might have heard of someone who can direct you. Send him an email to grab his attention; I'm pretty sure he won't mind me mentioning him.
I do recall a few Bone-Primary patients here, but do not recall whether their's was indolent or the conventional aggressive strains. I hope the write you also.
max
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Thank you for that prompt response, Max!Maybe
PBL,
Your case is about as unusual as I have read, but our unofficial Rare Cancers person here, Po18Guy, might be able to mention some specialists or Rare Cancers Centers to you. He is expert with T-cell diseases and Stem Cell transplantation issues, among others, but might have heard of someone who can direct you. Send him an email to grab his attention; I'm pretty sure he won't mind me mentioning him.
I do recall a few Bone-Primary patients here, but do not recall whether their's was indolent or the conventional aggressive strains. I hope the write you also.
max
I will follow your advice and send a message to Po18Guy and hope for some answers.
I am grateful for your prompt response, which already helps somehow.
PBL
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Rare presentation, but a well-known lymphoma
I am a T-Cell guy, but may know a little about other forms. Sorry to hear of your concern, but very happy to hear of your remission. Lymphoma may both occur and relapse anywhere in your body, so standard vigilance and monitoring would be appropriate. It sounds as though it was in your marrow and may have migrated to, or invaded the bone? Did they consider radiation at all? As to maintenance Rituxan (a 'monoclonal antibody' drug), you have an option now if the lymphoma finds its way around the Rituxan. There is a more potent monoclonal antibody available: Ofatumumab (Arzerra). It is labelled for chronic lmymphocytic leukemia (CLL), but is now in trials for lymphomas.
It eliiminates more B-Cells than Rituxan, for a longer period of time, and eliminates those B-Cells which have a much lower surface expression of the protein CD20, which is the target of Rituxan. I have just recently received it in a clinical trial against Graft-Versus-Host-Disease, associated with allogeneic stem cell transplants.
If your follicular was limted to the bone (and it may have been), there is the potential that you will be in a very long-term remission - perhaps even for life. In the case of an inolent lymphoma, this is a true blessing. Praying that it does not relapse, but you have options if it ever does. As to the rarity of its presentation, the type of lymphoma is much more important than where it presented itself.
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Po18Guy, Thank you for your insightspo18guy said:Rare presentation, but a well-known lymphoma
I am a T-Cell guy, but may know a little about other forms. Sorry to hear of your concern, but very happy to hear of your remission. Lymphoma may both occur and relapse anywhere in your body, so standard vigilance and monitoring would be appropriate. It sounds as though it was in your marrow and may have migrated to, or invaded the bone? Did they consider radiation at all? As to maintenance Rituxan (a 'monoclonal antibody' drug), you have an option now if the lymphoma finds its way around the Rituxan. There is a more potent monoclonal antibody available: Ofatumumab (Arzerra). It is labelled for chronic lmymphocytic leukemia (CLL), but is now in trials for lymphomas.
It eliiminates more B-Cells than Rituxan, for a longer period of time, and eliminates those B-Cells which have a much lower surface expression of the protein CD20, which is the target of Rituxan. I have just recently received it in a clinical trial against Graft-Versus-Host-Disease, associated with allogeneic stem cell transplants.
If your follicular was limted to the bone (and it may have been), there is the potential that you will be in a very long-term remission - perhaps even for life. In the case of an inolent lymphoma, this is a true blessing. Praying that it does not relapse, but you have options if it ever does. As to the rarity of its presentation, the type of lymphoma is much more important than where it presented itself.
Radiation was not an option, as the tumor basically extended in all of my left pelvis, from the pubic bone (where the fracture occured) to the ischiatic bone, all of the hip socket and up the iliac crest. Radiation would have caused too much damage to my descending colon and bladder.
What prompted the question as to the form of a possible relapse now is that I have been having movement-limitating pain in my right shoulder, and the collarbone in particular. I made an appointment (logically, I thought...) with the rheumatologist who took X-rays, a sonogram and ordered a bone scan. The radiograph was essentially normal, sonogram showed some swelling with fluid which the doc said he could see at clinical exam, and the bone scan showed two areas of increased uptake in my clavicle. With all of those results, he said that he felt it was inflammatory and that he could go on investigating, but that it would cause more problems than it would solve. He did not, however, name what it could be...
I figured that I would be having another scan next July as part of my standard follow-up, and that whatever it was would not likely have killed me before then, so it could wait.
But then, in order not to go on making appointments for "nothing" - and worrying myself sick in the process - I asked him if he could indicate what a recurrence would look like, and was shocked to hear him reply that he didn't know BECAUSE THOSE PATIENTS WITH PBL NEVER RETURNED TO HIM ONCE THEY WERE IN THE HANDS OF THE HEMATOLOGISTS. Now, I do know for a fact that he is extremely busy and dedicated, but I cannot help being disappointed in him on this point...
I really feel that I need to move on with my life and focus on other things than this whole episode, but I find it difficult to do so without feeling confident that I can tell the difference between something worth making an appointment and any ordinary pain. It may seem irrational, but reflects my case history.
PBL
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We tend to think every pain is a relapse
That is our human nature. However, what you had is not bone cancer, but lymphoma in the bone. The chance of it choosing bone elsewhere in your body seems to me to be extremely unlikely. A single mutated B-Cell migrated to your bone as part of its routine travels, cloned itself for some reason and grew in your bone into hundreds of millions of clones of itself. For one of those clones to have survived, and to have then taken up residence in another bone elsewhere is about as likely as your chances of winning the lotto, I would think.
Each and every pain you feel is referred to as "noise" inasmuch as it raises the specter of a relapse. But, our fears rarely materialize. What you will have to be aware of is that it could relapse, and anywhere in your body, from brain to eyeballs to pancreas to spleen. Lymphoma, being a tumor of the immune system, can occur anywhere in your body. Vigilance is the correct response.
Will it relapse? No one can say. The advantage is that new treatments are arriving regularly, so hope is increasing as each day, week, month and year pass.
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You know your body the bestPBL said:Vigilance it will have to be, then...
I guess that's what the systematic follow-up tests are all about. I'm not so good at keeping an eye on every boo-boo. I do hope they will be able to pick up its scent if and when it does return.
Thank you for your wisdom.
PBL
With a lymphoma history, doctor should take any concerns seiously. Allow prudence to take precedence over paranoia.
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Hi PBL, Just got back on the
Hi PBL, Just got back on the boards recently. Although the term PBL was never used to describe my NHL, as I told you in previous threads I too had the lymphoma only in the bone marrow of my right hip and pelvic bones. I finished bendamustine and rituxan in late October and my November PET scan showed "NED" no evidence of disease. I don't go back to the oncologist until March.
I asked about how I would know if it came back too and they were just as vague as your doctors were. I think they honestly can not tell you because as we all know now each case is different and as po18guy said the cancer could never come back or come back somewhere else in your body. It's enough to drive you mad, believe me I know. I continue to have aches and pains and stiffness throughout my body, but my doc did say it would take up to 12-18 mos post-treatment for my body to feel 'normal' again. I am attributing most of my pains to that in an effort to stay positive.
Oddly enough ,I too have had a great deal of right shoulder pain recently. At one my apmts my doc had said there "might be something in your shoulder too". It was never confirmed to be the lymphoma because to do that they would have to biopsy the bone marrow there (that is how they diagnosed the hip). I had a muscular injury in that shoulder prior to my diagnosis and I truly feel that is what caused that pain and not lymphoma. However, flash forward several months and my shoulder is bothering me again. I am going to the orthopedic to check it out, but am also worried so I messaged my oncologist about it. Waiting to hear what she says.
So....too late for a long story to be short, but my decision is to just take one day at a time. For me the cancer showed it self to me via intense, severe pain in my hip and so if I ever feel that kind of pain anywhere I will certainly be calling the doctor. For the rest of my somewhat normal pains I just manage through diet and exercise.
It's hard.
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AnyNailsJHS said:Hi PBL, Just got back on the
Hi PBL, Just got back on the boards recently. Although the term PBL was never used to describe my NHL, as I told you in previous threads I too had the lymphoma only in the bone marrow of my right hip and pelvic bones. I finished bendamustine and rituxan in late October and my November PET scan showed "NED" no evidence of disease. I don't go back to the oncologist until March.
I asked about how I would know if it came back too and they were just as vague as your doctors were. I think they honestly can not tell you because as we all know now each case is different and as po18guy said the cancer could never come back or come back somewhere else in your body. It's enough to drive you mad, believe me I know. I continue to have aches and pains and stiffness throughout my body, but my doc did say it would take up to 12-18 mos post-treatment for my body to feel 'normal' again. I am attributing most of my pains to that in an effort to stay positive.
Oddly enough ,I too have had a great deal of right shoulder pain recently. At one my apmts my doc had said there "might be something in your shoulder too". It was never confirmed to be the lymphoma because to do that they would have to biopsy the bone marrow there (that is how they diagnosed the hip). I had a muscular injury in that shoulder prior to my diagnosis and I truly feel that is what caused that pain and not lymphoma. However, flash forward several months and my shoulder is bothering me again. I am going to the orthopedic to check it out, but am also worried so I messaged my oncologist about it. Waiting to hear what she says.
So....too late for a long story to be short, but my decision is to just take one day at a time. For me the cancer showed it self to me via intense, severe pain in my hip and so if I ever feel that kind of pain anywhere I will certainly be calling the doctor. For the rest of my somewhat normal pains I just manage through diet and exercise.
It's hard.
Nails,
Any lymphoma can re-emerge as the same disease, as a different form of lymphoma, or as a form of leukemia very easily. The doctors are vague because so many differing possibilities are possible. It is honesty on their part. My hematologist told me at the end of chemo with PETS showing NED that
"I might never have cancer again, I might some day have the same strain of HL again, I might develop an aggressive form of NHL later, or I might get leukemia some day." ALL of these were clinically reasonable and possible. Chemo combos with radiation significantly increase the statistical liklihood of a lymphoma morphining into a leukemia, so I always refused radiation later, when I got prostate cancer.
You are doing as much as a person can do. Live and hope for the best, ask questions when it is reasonable to do so. My strain of indolent HL has a 15 % relapse rate, relatively high. What does that mean to me ? It means that I have an 85% liklihood to NOT relapse -- a number I like.
max
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New pain...
Hello again NailsJHS,
Happy to resume our conversation, but sorry to find that your recovery causes you some concern. Intriguing how we should both have shoulder issues after having both had hip pain... Unlike you, however, nothing showed in my shoulder at diagnosis over a year ago. I had had pain in the same shoulder in 2014, and, just like with my hip, the x-rays then revealed nothing.
In my quest for information, I read that as many as 20-25% of indolent lymphomas experience spontaneous remission, which might explain my personal experience with this disease...
The hematologist whom I saw last week told me she would order a PET scan for me, maybe next visit (in another eight weeks) - she was't quite sure what to do and seemed to want to discuss my case with the rhumatologist as well as her colleagues.
Do keep me posted on your own developments.
Wishing you the best,
PBL
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Hello PBL,
I am new to this community and am seeking any information I can find on PBL. I have a daughter in her 20s that was recently diagnosed with follicular lymphoma (grade 3a, stage 1 to 2, and BCL2 negative), and it was only found in her right humerus bone. As you said, this is extremely rare, so I feel so lucky to have happened upon your post. Her bloodwork and bone marrow biopsy were normal. Since her disease was only in that one bone and not systemic or found in any lymph nodes, her oncologist recommended radiation only. She received treatments totaling 24 Gray (12 sessions of 2 Gray each). Her six month PET follow-up showed no evidence of disease. I'd like to know how you are doing and what your scanning schedule has been like. We have had a second opinion and have conflicting recommendations on how often to do the scans. One doctor says every 6 months, and the other doctor said to do it yearly to decrease the amount of radiation she receives since she is so young and will have a lifetime of scanning ahead of her. Also, one doctor recommended a CT over the PET since it is a third of the amount of radiation as a PET. Can you offer any guidance?
Thank you so much,
Ann8
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Hello po18guy,
I just sent this same post to PBL but saw that you had conversed with him concerning his diagnosis, and since my daughter has a similar diagnosis, I thought I would touch base with you. I am new to this community and am seeking any information I can find on PBL. I have a daughter in her 20s that was recently diagnosed with follicular lymphoma (grade 3a, stage 1 to 2, and BCL2 negative), and it was only found in her right humerus bone. As PBL said, this is extremely rare, so I feel so lucky to have happened upon your posts. Her bloodwork and bone marrow biopsy were normal. Since her disease was only in that one bone and not systemic or found in any lymph nodes, her oncologist recommended radiation only. She received treatments totaling 24 Gray (12 sessions of 2 Gray each). Her six month PET follow-up showed no evidence of disease. I'd like to know if you have any new information you could share with me concerning her diagnosis. We have had a second opinion and have conflicting recommendations on how often to do the follow-up scans. One doctor says every 6 months, and the other doctor said to do it yearly to decrease the amount of radiation she receives since she is so young and will have a lifetime of scanning ahead of her. Also, one doctor recommended a CT over the PET since it is a third of the amount of radiation as a PET. Can you offer any guidance?
Thank you so very much,
Ann8
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Hello Ann8,
Sorry to see you join the "club", but glad to read overall good news regarding your daughter's response to treatment thus far. I was more than double her age at diagnosis and was given chemotherapy and two-year maintenance essentially because my lesions were not limited to "just one" and my response to treatment was rather, shall we say, sluggish. My lesions initially did not appear to change in size and very gradually reduced over the first few years post treatment, and I never got lower than Deauville 3, which is considered remission but "on the fence", after chemotherapy was completed. I was therefore closely watched for a few years, with semestrial MRIs and CTscans. I also had a couple of PET scans when pain flared up. Nevertheless, and despite having documented "rogue" cells in my peripheral blood over a year after completing maintenance, I have not yet needed any further treatment and have "graduated" to yearly CTscans; I still see my hematologist every six months with bloodwork.
This being a "chronic" cancer - which means it will need to be monitored for a long time - if your daughter has no particular cause for concern she might as well look to limit her exposure to radiation. That being said, the dose of radiation for a CT or even a PET scan is minimal nowadays. It is also possible to avoid those technologies altogether if she can get her hematologist to order MRIs. However, she will likely meet some resistance there, as whole-body MRIs take longer than CTs and are therefore costly.
Some patients / doctors also agree - once stabilized - to have imaging done only when experiencing symptoms (or doubts).
I hope this answers your questions. Please feel free to come back for more if need be.
PBL
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PBL,
Thank you so much for your response. I hate that you didn't have the greatest response to treatment, but I am glad you are at least managing it well. Do you have any idea as to what might have caused yours to develop in the first place?
We were told that the area in her arm would enhance on an MRI for quite some time since there is still inflammation there from the lesion and radiation. So, since that wasn't a reliable scan in her case, we did the PET scan. We were told the MRI could light up in that area for several years. I will have to revisit this topic with her oncologist at her next appointment. An MRI does sound a lot better than receiving any radiation.
Thank you for your kind response and guidance!
Ann8
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Ann8,
You are very welcome. I must say I didn't spend too much time speculating over the causes of my disease; there might have been some clear-ish leads if I had been raised on a farm, had had Agent Orange exposure or had used pesticides myself…
Your daughter's doctors saying that post-treatment MRIs could be misleading matches my own experience, which brings me back to my previous remark on "once stabilized": she might agree to getting CT scans (or even PET scans) for the first couple of years, and once her disease's behaviour is ascertained, move to a yearly - or even occasional - CT. She might also raise the question of checking her peripheral blood for MRD (minimal residual disease), as that could be useful in defining the depth of her response, and so the frequency of future imaging.
Kind regards to you both.
PBL
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PBL,
Yes, I question whether pesticide exposure could have done it for my daughter. We used to spray our yard when she was little. I also question whether her long-term use of minocycline for acne could have had something to do with it. Interestingly, I read where trauma to the area can sometimes trigger it due to long-term inflammation. She popped her arm pretty badly while exercising just prior to developing pain in that arm. I have wondered if the incident with the popping while weight lifting could have caused it or not. It very well could have been there before that. I hate that we will probably never know.
Ok, your remark about "once stabilized" makes sense. And, wonderful point about the MRD blood check! Thank you so much! I will definitely speak to her doctor about this. Thank you again for speaking with me!
All the best,
Ann8
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Just saw this. My apologies. Bear with me if you will. I will begin with the big picture. Each of us having absolutely unique DNA, we are individuals. We are all of the same species, but each of us possesses a singular identity within the species. Cancer involves a mutation of our unique DNA in one type of our body's cells and thus might be considered the most personal of diseases. Even though we may have the same type of cancer, our personal DNA is at the core of it all. It is a disturbance in our DNA which drives the formation of a single cancer cell, which then multiples at an abnormal rate. Another factor is where the cancer decides to begin multiplying. Lymphoma is a cancer which can begin anywhere we have blood cells.
Primary bone lymphoma is unusual, but possible only because our blood circulates in our bones - thus allowing bone growth as we mature. For some reason which cannot be known yet, a single lymphocyte in your daughter's bone developed a flaw in its DNA when it replicated itself. Our immune system identifies and kills defective, even cancerous cells on a daily basis. Occasionally, a single cell avoids detection and begins cloning itself. As well, cancer has the devilish ability to tell your body's immune system to leave it alone.
Cancer in the bone is somewhat akin to cancer in the central nervous system, in that its location makes treating it more of a challenge. It remains treatable, but some unique manner of addressing it is necessary. Radiation is sometimes necessary, as there my be little other means of reaching the tumor cells. Sadly, radiation causes some level of damage to each cell it penetrates. It can cause flaws in cellular DNA which might be benign, or which may sooner or later develop into yet another cancer. It is truly a double-edged sword.
In your daughter's case, she is young and cellular damage is far more critical to avoid than in the case of an 80 year old. Imagine that radiation damage might lead to another cancer in 20 years. A definite cause of concern to someone in their 20s, but essentially irrelevant to an 80 year old. I believe treatment in the young might be intentionally limited to some extent, trading less damage for a slight increase in a future recurrence. Your daughter has 60 more years to deal with damage from treatment, so the bias might be to limit that damage for the sake of quality of life. At the same time, medical science is constantly advancing and less toxic/more effective treatments are arriving on a steady basis.
For the above reasons, the level of radiation needed for a PET versus CT scans is more important in the young than the older. As to scanning frequency, a balance must be struck between early relapse detection and the damage caused by increased and repeated radiation damage. In this arena, there are no firm answers, only general principles. You have noted that doctors have differing views. This points out their different experience and the unpredictable nature of the disease. At some point, we must make a leap of faith and live with the result. It is far better not to look back and second guess - that will lead to unending anxiety.
The last I heard, relapses are caught much more frequently via the appearance of symptoms than they are by scanning. As well, the threshold of detection by scanning is something on the order of one million cancer cells. In that regard, the limitation of scanning may be easily observed. One could be scanned monthly, but the likelihood of the radiation actually causing cancer far exceeds any benefit of the scan itself. Scanning is a wonderful tool in diagnostics, but has definite limitations and inherent dangers.
I was much older when all of this struck, so scanning was far less of a concern to me. I have lost count, but have received something on the order of 40 CT scans and more than a dozen PET scans. One of my cancers may have been caused by the scanning, but there is no way of knowing that.
As to your daughter, the younger she is, the more resilient her body is and the more likely she is to heal well. Time is our friend here, as research is ongoing. Although the subject matter is not the most pleasant, studying cancer is knowledge and knowledge is power. I must mention the importance of clinical trials here. I have participated in four trials, once of which placed me in remission long enough for newer therapies to be available. Medical science cannot advance without clinical trials, so they are something to investigate now, as well as when and if a relapse is ever suspected.
I wish I could be more specific, but the best we can do is enjoy each day of life we are given.
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Po18guy,
Thank you so very much for your reply. Your willingness to help others that you don't even know speaks volumes about your character. I truly appreciate the information you have shared.
It sounds like you have really been through it with this disease, and I am so sorry. I know it must take a toll on you, but I am inspired by your commitment to research in order stay informed on the latest trials and treatments.
I have done extensive research on my daughter's condition to the point that it became an obsession and was ultimately unhealthy for me and my whole family. I haven't been researching as much lately, but I do need to get back into it and maybe just allot a certain amount of time for it each day. In the beginning, I would research relentlessly for hours on end and let all other obligations go. It just wasn't healthy. I have a binder full of notes, and I think, at times, I drive her doctors a little crazy with all of my questions. I definitely agree that knowledge is power though.
I'm not certain the radiation was the right treatment for her, but we were steered in that direction because you could target just the arm with the radiation versus targeting the whole body with chemo or immunotherapy when the disease seemed to be localized. After reading your message, it seems that you could also lump radiation into the category of treatments that affect the whole body since you do run the risk of developing leukemia at a later date.
Also, I didn't realize that there needed to be around a million cancer cells for it to be detected. That makes complete sense though considering the size of a cancer cell.
It is important, like you said, to make the best decision you can at the time and to not look back. I struggle with that a lot.
Thanks again!
Ann8
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Happy to provide any help that I can. Lymphoma almost anywhere in the body is easily accessed by treatment, which flows in the same blood and lymph. However, the spinal column, brain and bone present challenges. There is just not that much blood flowing in the affected bone, and that is the only standard method of applying therapy. Radiation cannot be stopped and in areas where there is limited blood or lymph flowing, it may be the best choice.
There will always be a hematologist here or there who would suggest a different treatment. What they cannot predict, is if it will be effective. All cancer therapy causes damage of some sort. Various lymphoma therapies cause heart and lung damage, and a few cause potentially permanent nerve damage.
So, live for today and look forward. What has passed is something to learn from, not to re-visit often. If you are an anxious person by nature (1 in 5 Americans suffer from some form of anxiety) it might be beneficial to consider therapy, which can we very effective. The gold standard of anxiety treatment is called cognitive behavioral therapy. it is drug-free and can even be done over the phone. It basically teaches us to unlearn the thought processes which produced the anxiety. Just a thought.
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