I have been relapsing for some time with a light chain myeloma clone I have developed. My kappa light chain numbers have been in the thousands for many months now.
It is my understanding that all these light chains circulating in the blood can form protein plaques on internal organs, causing complications – especially with the kidneys and heart. I believe the name for this is amyloidosis.
I have read that dialysis does not remove kappa light chains form the blood. I have found little information on whether or not any protein plaques that develop from kappa light chains will dissolve in time if the disease and kappa light chains are brought down to normal levels, or if they are permanent.
It seems that the primary treatment, which makes sense, is to destroy the source of the light chains, the plasma cells producing them.
But if that is not successful, is there anything that can be done to reduce or remove the plaques that may develop?
And if there are plaques that develop, do they eventually dissolve or not and, if they do, how long does it take? Weeks, months years?
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Re: Kappa amyloid deposits - are they permanent?
Hi Eric,
I'm not 100% positive I have this right, but I will give it a shot. Please verify all this with your doc and others on this forum.
Light chain multiple myeloma is distinct from amyloidosis. Amyloidosis causes the formation of misfolded amyloid proteins that can in turn deposit in various organs throughout your body. Multiple myeloma (including light chain multiple myeloma) does not do this. There is also light chain deposition disease (LCDD), which can deposit non-amyloid light chains throughout one's body. But LCDD is also distinct from amyloidosis and it is also distinct from light chain multiple myeloma.
Yes, excess light chains (as well as excess calcium) that are result of light chain multiple myeloma (or "regular" multiple myeloma) can wreak havoc with your kidneys if left untreated. But I wouldn't assume you are experiencing organ damage specifically from amyloid deposition or that organs other than your kidneys (such as your heart) are potentially being directly impacted by your excess kappa free light chains unless you've been specifically diagnosed with amyloidosis.
I assume you are on top of your kidney function markers (creatinine, BUN, eGFR, etc) if your kappa is running high? Are these markers deteriorating? I believe the only way to prevent the potential kidney damage due to excess light chains in multiple myeloma patients is through the direct treatment of the myeloma disease (drug or transplant), thereby lowering your involved FLC level. Indirect measures such as reducing calcium levels through bisphosphonate use or taking some of the burden off of your kidneys via dialysis may help overall kidney function, but a high FLC level still needs to ultimately be addressed through treatment.
The good news for patients in general is that depending on the type of kidney damage (cast nephropathy, etc) that one may have experienced from multiple myeloma and associated excess free light chains, that damage may be reversed via multiple myeloma treatment.
I hope you find a treatment that can work for you. If you aren't having any success with your current doctor's suggested regimens, have you considered getting a second opinion elsewhere?
I'm not 100% positive I have this right, but I will give it a shot. Please verify all this with your doc and others on this forum.
Light chain multiple myeloma is distinct from amyloidosis. Amyloidosis causes the formation of misfolded amyloid proteins that can in turn deposit in various organs throughout your body. Multiple myeloma (including light chain multiple myeloma) does not do this. There is also light chain deposition disease (LCDD), which can deposit non-amyloid light chains throughout one's body. But LCDD is also distinct from amyloidosis and it is also distinct from light chain multiple myeloma.
Yes, excess light chains (as well as excess calcium) that are result of light chain multiple myeloma (or "regular" multiple myeloma) can wreak havoc with your kidneys if left untreated. But I wouldn't assume you are experiencing organ damage specifically from amyloid deposition or that organs other than your kidneys (such as your heart) are potentially being directly impacted by your excess kappa free light chains unless you've been specifically diagnosed with amyloidosis.
I assume you are on top of your kidney function markers (creatinine, BUN, eGFR, etc) if your kappa is running high? Are these markers deteriorating? I believe the only way to prevent the potential kidney damage due to excess light chains in multiple myeloma patients is through the direct treatment of the myeloma disease (drug or transplant), thereby lowering your involved FLC level. Indirect measures such as reducing calcium levels through bisphosphonate use or taking some of the burden off of your kidneys via dialysis may help overall kidney function, but a high FLC level still needs to ultimately be addressed through treatment.
The good news for patients in general is that depending on the type of kidney damage (cast nephropathy, etc) that one may have experienced from multiple myeloma and associated excess free light chains, that damage may be reversed via multiple myeloma treatment.
I hope you find a treatment that can work for you. If you aren't having any success with your current doctor's suggested regimens, have you considered getting a second opinion elsewhere?
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Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Kappa amyloid deposits - are they permanent?
Amyloidosis only occurs when the free light chains produce insoluble deposits (amyloid). In most cases of light chain myeloma, this is not a problem. In any case, it is easy to test for with a simple biopsy.
What does your doctor say?
What does your doctor say?
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Davidg - Name: David
- When were you/they diagnosed?: Feb 2015 - AL Amyloidosis
- Age at diagnosis: 53
Re: Kappa amyloid deposits - are they permanent?
Hi Eric
Not a doctor, but have been through this twice with my husband. Not an issue for us right now, so I am relying on memory. Davidg's explanation concurs with what we have been told regarding amyloids.
Excess light chains can be absorbed. Rationale for treatment is the lower activity level of the myeloma means a lesser production of the light chains. They can be absorbed by the kidneys, but it takes more time. Half life is around 2-3 hours for kappa light chains; 4-6 hours for lambda. Serum free light chain tests have a pretty wide margin of error (up to 20%). If your numbers are in the thousands (assume that's per liter), a big swing is necessary to have the change be significant.
Kappas are smaller than lambdas. Directionally, kappa is associated with light chain deposition disease. Lambda is associated with amyloidosis. Fat pad biopsy with Congo red stain test will give 70% (or thereabouts) confirmation on presence or absence of amyloid. Gold standard if kidney is an issue is a kidney biopsy.
Plasmapheresis is one way to strain off the excess light chains. Depending on medical center, it's a band-aid and they don't like to / won't do it, or it's an opportunity to get your numbers down to hit the myeloma harder with treatment. I very recently read that high cut-off dialysis can also capture some of the excess light chains. At present, dialysis is not required, so I don't know anything more about it. Try a google search.
Hope that's helpful and wishing you all the best
Not a doctor, but have been through this twice with my husband. Not an issue for us right now, so I am relying on memory. Davidg's explanation concurs with what we have been told regarding amyloids.
Excess light chains can be absorbed. Rationale for treatment is the lower activity level of the myeloma means a lesser production of the light chains. They can be absorbed by the kidneys, but it takes more time. Half life is around 2-3 hours for kappa light chains; 4-6 hours for lambda. Serum free light chain tests have a pretty wide margin of error (up to 20%). If your numbers are in the thousands (assume that's per liter), a big swing is necessary to have the change be significant.
Kappas are smaller than lambdas. Directionally, kappa is associated with light chain deposition disease. Lambda is associated with amyloidosis. Fat pad biopsy with Congo red stain test will give 70% (or thereabouts) confirmation on presence or absence of amyloid. Gold standard if kidney is an issue is a kidney biopsy.
Plasmapheresis is one way to strain off the excess light chains. Depending on medical center, it's a band-aid and they don't like to / won't do it, or it's an opportunity to get your numbers down to hit the myeloma harder with treatment. I very recently read that high cut-off dialysis can also capture some of the excess light chains. At present, dialysis is not required, so I don't know anything more about it. Try a google search.
Hope that's helpful and wishing you all the best
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philatour - Who do you know with myeloma?: spouse
Re: Kappa amyloid deposits - are they permanent?
Hi Eric,
It is nice to hear from you again! I am sorry you relapsed. I cannot speak about kidneys, but I will tell you about my recent experience. Last fall I relapsed and the darn disease reached my stomach. The tests showed amyloidosis there, a green sticky substance (bleah). I stopped eating all together for one week and was in a bad shape.
In the same days I started Revlimid after being off medicines for two years, and soon got better. My doctor told me that while the production of amyloidosis stopped, the deposit of substance which was already there would remain. However, now I am fine and have no problems with digestion. I suppose it is a matter of quantities then.
I wish you all the best - ciao ciao
It is nice to hear from you again! I am sorry you relapsed. I cannot speak about kidneys, but I will tell you about my recent experience. Last fall I relapsed and the darn disease reached my stomach. The tests showed amyloidosis there, a green sticky substance (bleah). I stopped eating all together for one week and was in a bad shape.
In the same days I started Revlimid after being off medicines for two years, and soon got better. My doctor told me that while the production of amyloidosis stopped, the deposit of substance which was already there would remain. However, now I am fine and have no problems with digestion. I suppose it is a matter of quantities then.
I wish you all the best - ciao ciao
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Annamaria - Name: Annamaria
- Who do you know with myeloma?: I am a patient
- When were you/they diagnosed?: April 2012
- Age at diagnosis: 58
Re: Kappa amyloid deposits - are they permanent?
Great responses thus far!
Eric, it is true that free light chains can misfold into amyloid, causing direct organ toxicity. This does not usually happen, but attention needs to be paid to the possibility. If there is organ dysfunction typical of amyloid (albumin losses in the urine, thickening of the heart with heart failure symptoms, carpal tunnel syndrome, drop in blood pressure - or dizziness - with standing), then the doctor should investigate for amyloid.
This can be accomplished by a simple fat pad biopsy, or, if negative with high suspicion, a direct organ biopsy. It is important to make this diagnosis because patients with amyloid require subtly different management – for example, they tend to tolerate multiple myeloma drugs a bit differently.
In terms of the other part of your question, the amyloid deposits generally 'stay', but the organs can remodel around them and function can improve. Conventional management is with therapy targeting the clone producing the 'bad' light chains. There is, however, a research focus on amyloid resorbing treatments, and NEOD001 (from Prothena) is furthest along in my opinion. Hopefully this (or another like drug) will get approved in the near future.
Hope this helps!
Eric, it is true that free light chains can misfold into amyloid, causing direct organ toxicity. This does not usually happen, but attention needs to be paid to the possibility. If there is organ dysfunction typical of amyloid (albumin losses in the urine, thickening of the heart with heart failure symptoms, carpal tunnel syndrome, drop in blood pressure - or dizziness - with standing), then the doctor should investigate for amyloid.
This can be accomplished by a simple fat pad biopsy, or, if negative with high suspicion, a direct organ biopsy. It is important to make this diagnosis because patients with amyloid require subtly different management – for example, they tend to tolerate multiple myeloma drugs a bit differently.
In terms of the other part of your question, the amyloid deposits generally 'stay', but the organs can remodel around them and function can improve. Conventional management is with therapy targeting the clone producing the 'bad' light chains. There is, however, a research focus on amyloid resorbing treatments, and NEOD001 (from Prothena) is furthest along in my opinion. Hopefully this (or another like drug) will get approved in the near future.
Hope this helps!
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Dr. James Hoffman - Name: James E. Hoffman, M.D.
Beacon Medical Advisor
Re: Kappa amyloid deposits - are they permanent?
Thank you everyone for your responses, especially Dr. Hoffman. The Beacon is such a good resource to get information from a top myeloma specialist. I did a lot of searching for information about this and found there just was not a lot out there or I would need a medical degree to understand it. I feel a lot better now knowing more about what light chains do in the body and their potential hazards and what to look out for. I brought this up with my oncologist but he did not seem that concerned about the high light chains. It may be he did not have much experience with complications from high light chains.
I started treatment with daratumumab two weeks ago and hopefully this will stop and reverse the progression of my myeloma. I feel it may be working because my back pain has shown some improvement in the last few days. But I will have to wait for lab numbers to know for sure. Best wishes to everyone.
I started treatment with daratumumab two weeks ago and hopefully this will stop and reverse the progression of my myeloma. I feel it may be working because my back pain has shown some improvement in the last few days. But I will have to wait for lab numbers to know for sure. Best wishes to everyone.
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Eric Hofacket - Name: Eric H
- When were you/they diagnosed?: 01 April 2011
- Age at diagnosis: 44
Re: Kappa amyloid deposits - are they permanent?
Hello, Eric.
I just wanted to say good luck to you with the daratumumab. I hope it works very well for you. Good luck.
Regards
I just wanted to say good luck to you with the daratumumab. I hope it works very well for you. Good luck.
Regards
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JPC - Name: JPC
Re: Kappa amyloid deposits - are they permanent?
I have multiple myeloma and amyloidosis with cardiac involvement, so I can share what happened with me.
I had some serious amyloid deposits in my heart and it was causing some major shortness of breath. The walls of my heart had thickened substantially (shown through heart MRIs), causing it to work much harder than normal.
First step was to stop any new deposits from forming, which happens to be the same treatment as multiple myeloma. For me, this was Revlimid, Velcade, and dex (in 2011). Unfortunately they don't really have any way to remove the amyloid once it's there, but the thought (unproven really) was that my immune system, over time, could remove them. I had 16 weeks of that treatment followed by tandem stem cell transplants. All worked great to get me into complete remission.
The better news was that my immune system did do its job and after a year or so virtually all amyloid from my heart was gone and my heart was normal in size and function. My cardiologist said if he hadn't known my history he would have never known I had any issues. He also mentioned that had I been in California with some of his colleagues at Stanford, that they would have pushed for a heart transplant initially (it was that bad).
So, yes, I'm living proof the body can get rid of the amyloid on its own, but you need to control new deposits from forming first.
I had some serious amyloid deposits in my heart and it was causing some major shortness of breath. The walls of my heart had thickened substantially (shown through heart MRIs), causing it to work much harder than normal.
First step was to stop any new deposits from forming, which happens to be the same treatment as multiple myeloma. For me, this was Revlimid, Velcade, and dex (in 2011). Unfortunately they don't really have any way to remove the amyloid once it's there, but the thought (unproven really) was that my immune system, over time, could remove them. I had 16 weeks of that treatment followed by tandem stem cell transplants. All worked great to get me into complete remission.
The better news was that my immune system did do its job and after a year or so virtually all amyloid from my heart was gone and my heart was normal in size and function. My cardiologist said if he hadn't known my history he would have never known I had any issues. He also mentioned that had I been in California with some of his colleagues at Stanford, that they would have pushed for a heart transplant initially (it was that bad).
So, yes, I'm living proof the body can get rid of the amyloid on its own, but you need to control new deposits from forming first.
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RayGunter - Name: Ray Gunter
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Sept 2011
- Age at diagnosis: 38
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