[Covelo]

MGUS with suspected amyloidosis

by Covelo on Sat Mar 16, 2013 8:21 pm

Hi. I have known I had MGUS since 2005. We have been monitoring my m-protein (IgG lambda type) over that time and it has been consistently falling from its original value of 1.8 g/dl. Now at 1.3. My freelites have been normal and consistent over this same time period. Total IgG has stayed at 2.1 g/dl over the same period. I developed proteinuria last year and a kidney biopsy detected possible amyloids. I say possible because they screwed up the immunofluoresence stain so could only suggest lambda type amyloids. Fibrils were observed via electron microscopy though. My questions are, does the amyloid mean that my MGUS might be progressing to multiple myeloma. I get my bone marrow results on Monday. Also how much do m-protein levels vary. Seems like a good thing that mine are going down but... At first I thought I was just pissing it away but the amount of IgG in my urine is very small compared to my serum levels. Thanks

[Dr. Peter Voorhees]

Re: MGUS with suspected amyloidosis

by Dr. Peter Voorhees on Mon Mar 18, 2013 12:13 am

Your situation does sound suspicious for amyloidosis. If there is any question as to whether or not this is AL amyloidosis (in your case caused by lambda light chain amyloid deposits), you could have your biopsy tissue sent to the Mayo Clinic for Mass Spectroscopy-based protein sequencing. They will essentially sequence the amyloid protein and tell you if it is a lambda light chains or something else. This might be worth considering, especially in light of the normal serum free light chains (which is unusual but not impossible in amyloidosis).

I doubt you have myeloma based on what you have described, but the marrow biopsy will give us more information about that. And your theory about voiding away the M-protein may actually be true. I have seen M-spikes (along with albumin and other serum proteins) drop in the context of developing nephrotic syndrome from amyloidosis.

Let us know how things go. Hang in there!

Pete V.

[Covelo]

Re: MGUS with suspected amyloidosis

by Covelo on Mon Mar 18, 2013 10:21 pm

Thanks Dr Voorhees,

Marrow biopsy came back with 10% clonal plasma cells so right on the tipping point for progression. chromosome analysis came back with t(4:14) translocation and 13q14.3 deletion so not what I was hoping to see.

Still the amyloid in my kidney is the primary concern. My oncologist wants to proceed with a CyBorD regimen while mainly monitoring protein levels in urine for stability. With my free lambda levels currently at 25, is there any relevance to be had from monitoring for a decrease?

My main concern is regarding how definitive the diagnosis is for light chain amyloidosis considering my freelites are within the normal ranges and no results for specific immunoflourescent stains in the kidney biopsy (assay was messed up). They did see the congo red staining with the green bifurgence and fibrils under electron micro so likely definitive for amyloid. How confident should I be proceeding to treatment without definitively showing this is AL form and not hereditary with MGUS masking?

Thanks

[stann]

Re: MGUS with suspected amyloidosis

by stann on Tue Mar 19, 2013 4:27 pm

HI Covelo,
My post is sort of related, but possibly not very related...
I was diagnosed with multiple myeloma and primary amyloidosis. They tested me for AL amyloidosis because I was having throat issues which turned out to be something else.
The amyloidosis was diagnosed with a belly fat biopsy. This was in the fall of 2009 and I have been through a lot of treatment.
The specialists basically have said they'd treat the multiple myeloma. The only thing differently they'd do would be more careful in checking for organ issues (related to amyloidosis) before treatments, as some of the treatments are hard on different organs. Other than that, their target was my myeloma.
It's been 3 and half years, and the amyloidosis has not shown any signs, and I've been told that many times you'll test positive for it and it will never rear it's ugly head.
When first told I had systemic primary amyloidosis, I assumed the worst. But if it is associated with myeloma it is not as bad as primary amyloidosis by itself. I know this is a very non scientific explanation and it does not make a lot of sense to me, but that is my laymans, distilled version of my understanding.
Not sure if that is helpful or not, but since not many of us have both, thought I'd chime in.
Good luck to you.