I am posting a new thread in the hopes of getting further feedback on my situation. I discussed it earlier here:
"Light Chain Deposition Disease (LCDD) & Multiple Myeloma," Beacon forum discussion started May 5, 2013.
I have IgG lambda smoldering myeloma. Last tests showed IgG = 3160 mg/dl; lambda free light chain (FLC) level = 14.8 mg/dL; paraprotein (M-spike) = 2.14 mg/dL.
While I test positive for proteinemia, I am negative for proteinuria (I've had urine IFX and UPEP).
So, my nephrologist suspected there was a chance of Light Chain Deposition Disease, and I therefore had a kidney biopsy last week. My biopsy results are completely negative for LCDD and my kidneys look happy and healthy and all my renal markers are just dandy.
My nephrologist alternatively suspected some sort of strange electrical phenomena going on that causes my kidneys to repel my FLCs, but had no idea what that might be. I then educated him on the very rare possibility that the FLCs may be forming into tetramer configurations and are either physically too large to pass through the kidney or are polar in charge to the kidney cells, thereby being rejected by the kidney. He never heard of this, which doesn't surprise me as there are only a handful of cases in the world where this has occurred (google on "tetrameric bence jones" and also see http://www.ncbi.nlm.nih.gov/pubmed/6437210 )
I then asked the question of whether I should be concerned that these FLCs aren't getting flushed out through my kidneys and he wasn't sure about that either. Would the FLCs just self-destruct into harmless subcomponents or could they be accumulating in my gut or heart or elsewhere and should I be worried about this? I'm also going to talk to a couple of multiple myeloma specialists about this over the next couple of weeks, but I would love to hear from somebody that has some knowledge about this.
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Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Positive for proteinemia, negative for proteinuria?
Free light chains (FLCs) typically pass through the glomeruli in the kidneys and are resorbed in the proximal tubules where proteolysis breaks them down. They are small enough to pass through the glomeruli but do not typically show up in the urine since they are resorbed first. They show up in the urine when the level of the free light chains exceeds the capacity of the receptors at the proximal tubules that bind the FLC and bring it back into the kidney for decomposition.
http://www.wikilite.com/wiki/index.php/The_kidney_and_monoclonal_free_light_chains
Since your lambda levels are int he normal range and as you said your kidneys seem to be healthy, I would suspect that your FLCs are being produced and decomposed in a typical normal fashion.
http://www.wikilite.com/wiki/index.php/The_kidney_and_monoclonal_free_light_chains
Since your lambda levels are int he normal range and as you said your kidneys seem to be healthy, I would suspect that your FLCs are being produced and decomposed in a typical normal fashion.
Re: Positive for proteinemia, negative for proteinuria?
Hi Covelo, his lambda FLC's are outside the normal range. They are elevated at 148 mg/l (14.8 mg/dl). However, perhaps your theory is on target, since his kidneys are humming along beautifully.
Good luck, Multibilly!
Good luck, Multibilly!
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terryl1 - Name: Terry
- Who do you know with myeloma?: self
- When were you/they diagnosed?: August 10, 2011
- Age at diagnosis: 49
Sorry.Re: Positive for proteinemia, negative for proteinuria?
If there was direct damage to the kidney from light or heavy chains, this should be able to be seen on the immunohistochemistry in the kidney biopsy. Either amyloid deposition or light chain deposition should be easily identified. The urine finding in this case is usually striking albuminuria as opposed to dramatic increases in the monoclonal protein.
If the serum albumin and creatinine are normal and the urine does not show albuminuria either, this would make light chain deposition disease and renal amyloid unlikely.
The tetramer configuration is a possibility but it may be that your renal function is good and there is reabsorption of light chain with proteolysis that is then not detected on the urine immunofixation.
The accumulation of the light chain in other parts of the biopsy is always a concern. One of the easiest tests to do to evaluate for amyloid is to have prior bone marrow stained for amyloid with Congo red. Many times amyloid can be seen in the bone marrow blood vessels and this will "light up" with Congo red stain and birefringent microscope analysis.
I hope this makes sense.
If the serum albumin and creatinine are normal and the urine does not show albuminuria either, this would make light chain deposition disease and renal amyloid unlikely.
The tetramer configuration is a possibility but it may be that your renal function is good and there is reabsorption of light chain with proteolysis that is then not detected on the urine immunofixation.
The accumulation of the light chain in other parts of the biopsy is always a concern. One of the easiest tests to do to evaluate for amyloid is to have prior bone marrow stained for amyloid with Congo red. Many times amyloid can be seen in the bone marrow blood vessels and this will "light up" with Congo red stain and birefringent microscope analysis.
I hope this makes sense.
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Dr. Jason Valent - Name: Jason Valent, M.D.
Beacon Medical Advisor
Re: Positive for proteinemia, negative for proteinuria?
Thank you Dr. Valent. This all makes sense.
My serum creatinine and albumim are normal, Only a "faint albumin band on 100x concentration" showed up on one of my earlier urine fractionalization tests.
My kidney biopsy showed no deposits were present.
I reviewed my bone marrow pathology report from 11/2012 and I see no mention of congo red staining or "amyloid" ...but just about every other kind of staining technique was in there
I will discuss all this with my oncologist.
Thanks again!
My serum creatinine and albumim are normal, Only a "faint albumin band on 100x concentration" showed up on one of my earlier urine fractionalization tests.
My kidney biopsy showed no deposits were present.
I reviewed my bone marrow pathology report from 11/2012 and I see no mention of congo red staining or "amyloid" ...but just about every other kind of staining technique was in there
I will discuss all this with my oncologist.
Thanks again!
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Multibilly - Name: Multibilly
- Who do you know with myeloma?: Me
- When were you/they diagnosed?: Smoldering, Nov, 2012
Re: Positive for proteinemia, negative for proteinuria?
Question: What if you now show albumin in your IFE test, but the amyloid fat pad biopsy and marrow was negative? Should I assume everything is amyloid negative as well? What if you can't have a kidney biopsy due to a bleeding condition?
Also, would flank pain be a presentation of amyloid kidney? I am having some electrolyte imbalances now as well as elevated anion gap, elevated serum albumin, and a gradual decreasing GFR. Should I assume my urine protein is from some other cause ... HBP?
Also, would flank pain be a presentation of amyloid kidney? I am having some electrolyte imbalances now as well as elevated anion gap, elevated serum albumin, and a gradual decreasing GFR. Should I assume my urine protein is from some other cause ... HBP?
Re: Positive for proteinemia, negative for proteinuria?
Responding to bad blood,
If there is not another explanation for the albumin in the urine (i.e., hypertension, diabetes), then kidney biopsy would be the next step. Especially if the GFR is declining. It depends on how severe the bleeding condition is. If it is low factor X due to amyloid, that would potentially increase the risk of bleeding with kidney biopsy. If a kidney biopsy is not able to be done, the proteinuria will have to be managed with medication as best as possible (ACE inhibitors or angiotensin receptor blockers).
Flank pain would not be a typical symptom. Monoclonal proteins in the blood cause low anion gap (trivia question for medicine residents). Elevated serum albumin is not a bad thing.
If there is not another explanation for the albumin in the urine (i.e., hypertension, diabetes), then kidney biopsy would be the next step. Especially if the GFR is declining. It depends on how severe the bleeding condition is. If it is low factor X due to amyloid, that would potentially increase the risk of bleeding with kidney biopsy. If a kidney biopsy is not able to be done, the proteinuria will have to be managed with medication as best as possible (ACE inhibitors or angiotensin receptor blockers).
Flank pain would not be a typical symptom. Monoclonal proteins in the blood cause low anion gap (trivia question for medicine residents). Elevated serum albumin is not a bad thing.
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Dr. Jason Valent - Name: Jason Valent, M.D.
Beacon Medical Advisor
Re: Positive for proteinemia, negative for proteinuria?
I know one or two others here have already said this, but I want to thank you Dr. Valent for your reply to this and other questions that have been asked in the forum lately. I especially appreciate the time you've taken in many cases to explain the rationale for your feedback.
Re: Positive for proteinemia, negative for proteinuria?
Thank your Dr. Valent,
No, its not possible to have the biopsy. Very low factor VIII & von Willebrand levels. I have been having increased bleeding tendencies as well. My hpb is already being controlled by beta blockers. My GP suggested renal work up. I had my skin lesions biopsied this week and will know the results in a week. Hoping its nothing serious.
No, its not possible to have the biopsy. Very low factor VIII & von Willebrand levels. I have been having increased bleeding tendencies as well. My hpb is already being controlled by beta blockers. My GP suggested renal work up. I had my skin lesions biopsied this week and will know the results in a week. Hoping its nothing serious.
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