Hello Everyone,
My story started in 2011 with a stabbing pain in my shoulder. In 2012, I had countless tests, saw a rheumatologist, then a neurologist who decided I had chronic inflammatory demyelinating polyneuropathy (CIDP). IVIG treatment failed due to aseptic meningitis. During that year, an M-spike was discovered. After seeing an oncologist who repeated labs, CTs, scans, etc., everything came back normal.
I've been on medication since 2012 for severe neuropathy, which has only gradually worsened. The past 18 months I have been in SEVERE chronic pain throughout my body 24 hours a day (I am now in a wheelchair).
In July of this year I went for my annual check up with my neurologist, who informed me, "Your pain is not from the "CIDP". Due to the worsening symptoms, specialized bloodwork was ordered (it had been 4 years).
Well, my IgA (kappa) has tripled since 2012, My IgG and IgM are now below normal = back to the oncologist.
August 11, a bone marrow biopsy was performed and of course more labs and scans.
Abnormal Results; 7% plasma cells in bone marrow and apparently I am now anemic. Counts clear. etc.
.
My oncologist tells me normally he would take the "watch and wait" approach, BUT he is very concerned and thinks a plasma cell dyscrasia is causing the severe neuropathic pain. He has recommended I go to Dana-Farber in Boston.
Now I am confused and unsure what to do.
I live close to Syracuse, New York, I am 64 years old, married, forced to retire early and am now on SSDI.
That's my story. Thank you all who have read this. Your feedback is welcome.
Dennis
Forums
-
Dennis - Name: Dennis
- Who do you know with myeloma?: me
- When were you/they diagnosed?: March 2017
- Age at diagnosis: 64
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hi Dennis,
Welcome to the forum.
If it were me, I would go to Dana Farber and have the team there confirm or eliminate a diagnosis of a plasma cell dyscrasia and get their recommendation on appropriate next steps. Dana Farber has a world-class team for evaluating situations like yours.
I'm not a doc, but the combination of a 7% bone marrow plasma cell percentage, elevated IgA, suppressed IgM & IgG (immunoparesis), anemia and neuropathy strongly suggests the possibility of a plasma cell dyscraisa such as multiple myeloma or a related condition.
BTW, the bone marrow biopsy should have also included some other tests beyond a bone marrow plasma cell percentage count to help confirm what kind of a plasma cell disorder, if any, is present. If you have the biopsy report, it should have some sort of a summary paragraph that outlines what the pathologist thinks was the cause of your elevated bone marrow plasma cell percentage.
Lastly, do you have the results of any serum free light chain assays (kappa and lambda free light chain values in your blood)?
Welcome to the forum.
If it were me, I would go to Dana Farber and have the team there confirm or eliminate a diagnosis of a plasma cell dyscrasia and get their recommendation on appropriate next steps. Dana Farber has a world-class team for evaluating situations like yours.
I'm not a doc, but the combination of a 7% bone marrow plasma cell percentage, elevated IgA, suppressed IgM & IgG (immunoparesis), anemia and neuropathy strongly suggests the possibility of a plasma cell dyscraisa such as multiple myeloma or a related condition.
BTW, the bone marrow biopsy should have also included some other tests beyond a bone marrow plasma cell percentage count to help confirm what kind of a plasma cell disorder, if any, is present. If you have the biopsy report, it should have some sort of a summary paragraph that outlines what the pathologist thinks was the cause of your elevated bone marrow plasma cell percentage.
Lastly, do you have the results of any serum free light chain assays (kappa and lambda free light chain values in your blood)?
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Thought it was CIDP, but is it a plasma cell disorder?
I appreciate your response Mutibilly.
There is an addendum to the cytogenetic report that reads;
Plasma cell dyscrasia with abnormalities of TP53 and 1q21.3.
From what I can decipher, there were copies of 1q21.3 and 1p32.3. Also, a 5% deletion of tp53 (17P13.1).
Also says:
Monoclonal (kappa) cells with loss of CD19.
Kappa / lamba ratio 1.5.
CD4/CD8 ratio 2.9
This is all so confusing to me. I certainly do not want to start treatment if it isn't necessary. My oncologist did say the numbers weren't too awful, just that the neuropathy is throwing him off. as well as the IgA going from 295 to 1154 at some point in the last four years.? All of the CAT scans were normal.
There is an addendum to the cytogenetic report that reads;
Plasma cell dyscrasia with abnormalities of TP53 and 1q21.3.
From what I can decipher, there were copies of 1q21.3 and 1p32.3. Also, a 5% deletion of tp53 (17P13.1).
Also says:
Monoclonal (kappa) cells with loss of CD19.
Kappa / lamba ratio 1.5.
CD4/CD8 ratio 2.9
This is all so confusing to me. I certainly do not want to start treatment if it isn't necessary. My oncologist did say the numbers weren't too awful, just that the neuropathy is throwing him off. as well as the IgA going from 295 to 1154 at some point in the last four years.? All of the CAT scans were normal.
-
Dennis - Name: Dennis
- Who do you know with myeloma?: me
- When were you/they diagnosed?: March 2017
- Age at diagnosis: 64
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hi Dennis,
In addition to the excellent advice you've already received from Multibilly, I would just add that I think one possibility the doctors at Dana-Farber may explore is that you may have POEMS syndrome. This is something that is very often considered, I believe, in the presence of significant neuropathy and a monoclonal protein (M-protein or M-spike).
There is a quick, but very useful, posting about it by Dr. Valent here in the forum.
Also, Wikipedia has a useful article about POEMS, and there was an article about 4 years ago by a leading Mayo Clinic specialist about the diagnosis and treatment of the disease:
Dispenzieri, A., "How I treat POEMS", Blood, 2012 (full text of article)
I hope that your doctors are able to come to a proper diagnosis soon, and that they are able to successfully treat whatever it is that you have.
In addition to the excellent advice you've already received from Multibilly, I would just add that I think one possibility the doctors at Dana-Farber may explore is that you may have POEMS syndrome. This is something that is very often considered, I believe, in the presence of significant neuropathy and a monoclonal protein (M-protein or M-spike).
There is a quick, but very useful, posting about it by Dr. Valent here in the forum.
Also, Wikipedia has a useful article about POEMS, and there was an article about 4 years ago by a leading Mayo Clinic specialist about the diagnosis and treatment of the disease:
Dispenzieri, A., "How I treat POEMS", Blood, 2012 (full text of article)
I hope that your doctors are able to come to a proper diagnosis soon, and that they are able to successfully treat whatever it is that you have.
-
Jonah
Re: Thought it was CIDP, but is it a plasma cell disorder?
Thank you Jonah.
Yes, POEMS was discussed. My oncologist has ruled that out after clear imaging studies. Also said that POEMS is usually associated with an IgM spike, not IgA. He also said I was a "puzzling case". Great! I'm trying very hard to stay positive. Some days are difficult, though, when dealing with the severe pain.
Yes, POEMS was discussed. My oncologist has ruled that out after clear imaging studies. Also said that POEMS is usually associated with an IgM spike, not IgA. He also said I was a "puzzling case". Great! I'm trying very hard to stay positive. Some days are difficult, though, when dealing with the severe pain.
-
Dennis - Name: Dennis
- Who do you know with myeloma?: me
- When were you/they diagnosed?: March 2017
- Age at diagnosis: 64
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hi Dennis,
I think it is Waldenstrom's macroglobulinemia that is the non-multiple myeloma plasma cell disorder associated with an IgM M-spike.
POEMS, on the other hand, typically involves IgA or IgG M-spikes. Dr. Valent mentioned, for example, that IgA lambda is the most common type of POEMS monoclonal protein.
From your previous postings, you seem to have an IgA kappa M-spike, so that would make POEMS less likely, although it does not rule it out.
Just so you know, it looks like your M-spike is about 0.9 g/dL. That's not a high M-spike, by any stretch of the imagination. There are multiple myeloma patients who, at diagnosis, have M-spikes that are 6 or 7. But it's also not a trivially low, "could be a lab error" type of M-spike.
You say your kappa-lambda ratio was 1.5. Was this from a serum (blood) free light chain test, or some other kind of test?
The chromosomal abnormalities you listed are ones that are seen in people with multiple myeloma and its precursor diseases (smoldering multiple myeloma and MGUS). Apparently, though, the chromosomal abnormalities seen in people with POEMS are similar to those seen in myeloma patients. See this study:
http://onlinelibrary.wiley.com/doi/10.1111/ejh.12189/abstract
So I am not sure how much the abnormalities point to any specific diagnosis. Others here may be able to comment in more detail on that point, as well as the other aspects of the bone marrow biopsy report.
Good luck!
I think it is Waldenstrom's macroglobulinemia that is the non-multiple myeloma plasma cell disorder associated with an IgM M-spike.
POEMS, on the other hand, typically involves IgA or IgG M-spikes. Dr. Valent mentioned, for example, that IgA lambda is the most common type of POEMS monoclonal protein.
From your previous postings, you seem to have an IgA kappa M-spike, so that would make POEMS less likely, although it does not rule it out.
Just so you know, it looks like your M-spike is about 0.9 g/dL. That's not a high M-spike, by any stretch of the imagination. There are multiple myeloma patients who, at diagnosis, have M-spikes that are 6 or 7. But it's also not a trivially low, "could be a lab error" type of M-spike.
You say your kappa-lambda ratio was 1.5. Was this from a serum (blood) free light chain test, or some other kind of test?
The chromosomal abnormalities you listed are ones that are seen in people with multiple myeloma and its precursor diseases (smoldering multiple myeloma and MGUS). Apparently, though, the chromosomal abnormalities seen in people with POEMS are similar to those seen in myeloma patients. See this study:
http://onlinelibrary.wiley.com/doi/10.1111/ejh.12189/abstract
So I am not sure how much the abnormalities point to any specific diagnosis. Others here may be able to comment in more detail on that point, as well as the other aspects of the bone marrow biopsy report.
Good luck!
-
Jonah
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hi Dennis,
Jonah supplied some really good comments.
You said that "my oncologist has ruled that out [POEMS] after clear imaging studies." Did you have a PET/CT scan of your entire body? If not, what kind of imaging tests did you have? POEMS often occurs in the presence of osteosclerotic myeloma or plasmactyomas (either of which should be picked up by a PET/CT, but not necessarily by a skeletal xray survey).
Additionally, one could also make a case for amyloidosis with your specific symptoms (neuropathy & anemia) and lab values. A diagnosis of amyloidosis would require some additional specialized tests. Do you know if they did a Congo red stain on your bone marrow biopsy sample? A Congo red stain test may not reliably rule out amyloidosis with a negative stain result, but it is accurate for detecting amloidosis if it does yield a positive stain result.
In any case, it's pretty clear you have a plasma cell dyscrasia of some type based on your bone marrow biopsy and serum lab tests. Dana Farber is absolutely the kind of facility that has the right specialists to determine a specific diagnosis, whether it be MGUS, multiple myeloma, POEMS, amyloidosis, etc. Regardless which of the aforementioned diseases it turns out to be, the treatment options for all of these diseases tend to be pretty similar.
Jonah supplied some really good comments.
You said that "my oncologist has ruled that out [POEMS] after clear imaging studies." Did you have a PET/CT scan of your entire body? If not, what kind of imaging tests did you have? POEMS often occurs in the presence of osteosclerotic myeloma or plasmactyomas (either of which should be picked up by a PET/CT, but not necessarily by a skeletal xray survey).
Additionally, one could also make a case for amyloidosis with your specific symptoms (neuropathy & anemia) and lab values. A diagnosis of amyloidosis would require some additional specialized tests. Do you know if they did a Congo red stain on your bone marrow biopsy sample? A Congo red stain test may not reliably rule out amyloidosis with a negative stain result, but it is accurate for detecting amloidosis if it does yield a positive stain result.
In any case, it's pretty clear you have a plasma cell dyscrasia of some type based on your bone marrow biopsy and serum lab tests. Dana Farber is absolutely the kind of facility that has the right specialists to determine a specific diagnosis, whether it be MGUS, multiple myeloma, POEMS, amyloidosis, etc. Regardless which of the aforementioned diseases it turns out to be, the treatment options for all of these diseases tend to be pretty similar.
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Thought it was CIDP, but is it a plasma cell disorder?
Thank you, Multibilly, for your response.
Imaging studies were CT of chest, abdomen, and pelvis. I was a bit surprised more imaging / tests were not done.
Did not see any reference to a Congo stain on the bone marrow biopsy report. I too wonder about amyloidosis. To me, it did seem the doctor was quick to rule things out if I am such a "puzzling case".
Does anyone have an idea as to how long it will take to be seen at Dana-Farber? Weeks? Months?
Thank you
Imaging studies were CT of chest, abdomen, and pelvis. I was a bit surprised more imaging / tests were not done.
Did not see any reference to a Congo stain on the bone marrow biopsy report. I too wonder about amyloidosis. To me, it did seem the doctor was quick to rule things out if I am such a "puzzling case".
Does anyone have an idea as to how long it will take to be seen at Dana-Farber? Weeks? Months?
Thank you
-
Dennis - Name: Dennis
- Who do you know with myeloma?: me
- When were you/they diagnosed?: March 2017
- Age at diagnosis: 64
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hello everyone,
Instead of have me travel to Boston's Dana-Farber, my oncologist agreed to refer me to a closer facility, Wilmot Cancer Center in Rochester, New York. My appointment was on September 14. I was seen by 2 oncologists. I was told that they had not received all of my records and needed more time to study before giving my local oncologist any recommendations. Blood was drawn for more testing.
Today, the Rochester oncologist called. She explained that they requested the original bone marrow slides from my oncologist in order to do a Congo red stain? She said because of the severe neuropathy I may have amyloidosis.
I registered on their website / portal in order to see the September 14 lab results:
IgG - 456
IgA - 1254
IgM - 21
Kappa free light chains - 25.18
Lambda free light chains - 0.72
Kappa-lamba free light chain ratio - 34.97
Your input is greatly appreciated. Thanks so much,
Dennis
Instead of have me travel to Boston's Dana-Farber, my oncologist agreed to refer me to a closer facility, Wilmot Cancer Center in Rochester, New York. My appointment was on September 14. I was seen by 2 oncologists. I was told that they had not received all of my records and needed more time to study before giving my local oncologist any recommendations. Blood was drawn for more testing.
Today, the Rochester oncologist called. She explained that they requested the original bone marrow slides from my oncologist in order to do a Congo red stain? She said because of the severe neuropathy I may have amyloidosis.
I registered on their website / portal in order to see the September 14 lab results:
IgG - 456
IgA - 1254
IgM - 21
Kappa free light chains - 25.18
Lambda free light chains - 0.72
Kappa-lamba free light chain ratio - 34.97
Your input is greatly appreciated. Thanks so much,
Dennis
-
Dennis - Name: Dennis
- Who do you know with myeloma?: me
- When were you/they diagnosed?: March 2017
- Age at diagnosis: 64
Re: Thought it was CIDP, but is it a plasma cell disorder?
Hi Dennis,
Glad to hear your oncs are running a Congo red stain test. But note that since they are testing your bone marrow sample and not an affected organ, a negative test result will not definitively rule out amyloidosis. But it makes sense to start with testing the bone marrow sample since the sample is readily accessible.
If your oncs do make a diagnosis, I would still suggest going to a top facility and having any treatment plan put together by a multiple myeloma specialist. That treatment plan can always be administered by a local onc that is close to you.
Glad to hear your oncs are running a Congo red stain test. But note that since they are testing your bone marrow sample and not an affected organ, a negative test result will not definitively rule out amyloidosis. But it makes sense to start with testing the bone marrow sample since the sample is readily accessible.
If your oncs do make a diagnosis, I would still suggest going to a top facility and having any treatment plan put together by a multiple myeloma specialist. That treatment plan can always be administered by a local onc that is close to you.
-
Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
18 posts
• Page 1 of 2 • 1, 2