[AS_AM]

Significance of MGUS

by AS_AM on Sat Sep 28, 2013 1:57 pm

Hi.
Please help me Io understand the results on biopsy. As well I wish to understand what is my status MGUS or SMM. Do I need any anti-myeloma treatment? I am worry about nephrotoxic effect of FLC because I have signs of kidney damage. Age 46, male
1) Aspiration 11% of plasma cells.
2) IGA Lambda monoclonal band
3) Bence Jones positive
4) proteinuria 2.2 gr\24 on start of new anti-inflammatory treatment
5) IgA Lambda elevated up 150 -170 mg/l
BM BIOPSY ( taken from other site then aspiration )
"Biopsy of BM: Core 1.2 cm long. Cellularity: Slightly hyper cellular for age (60-70%). Cellularity: Slightly hyper cellular for age (60-70%). Red cells: Normoblastic. White cells: Complete maturation. Megakaryocytes: Normal Lymphocytes: No increase. Reticulin fibers: No increase. Collagen fibers: Absent. Hemosiderin: Absent. Other cells: Non obvious. Bone: Normal Other specific changes: None.
Immunostains: The CD138 and kappa and lambda light chains highlight mild increase (7- 12%) of mature-looking monoclonal plasma cells, lambda light chain predominant (lamda : kappa ratio =2/3:1). The CD56 and BCL1 are negative and the CD3 highlights not many reactive T lymphocytes. The CD20 is almost negative for B lymphocytes.
Conclusion: Slightly hyper cellular for age bone marrow with plasma cell dyscrasia.
There is only small amount of monoclonal plasma cells (less than 20%), therefore no diagnosis of myeloma can be made in this biopsy.
Clinical correlation is necessary.
Additional report: According to the physician's request, Congo red stain and immunostain for amyloid A were performed, and both of them are positive in the wall of a few blood vessels. "

On background Ankylosing Spondylitis ( kind of rheumatic disease). While evaluating low albumin and weight losing was found MGUS + secondary Amyloidosis approved by imunofixation of bone marrow.( Biopsy from rectum taken for Amyloidosis evaluation was positive as well ) Primary Amyloidosis was not ruled out by any scientific test, but by disease control achieved by doubling dose of anti-TNF blocker. I am use as well doxycycline -off label for potential anti amyloid activity.

[Dr. Adam Cohen]

Re: Significance of MGUS

by Dr. Adam Cohen on Wed Oct 02, 2013 5:45 pm

It sounds like you may have amyloidosis involving the kidney, especially if your proteinuria is mostly comprised of albuminuria. The question is which type of amyloidosis you have. Light-chain (AL) amyloidosis is caused by deposits of an abnormal immunoglobulin light chain that is being produced by abnormal plasma cells in the bone marrow, which you do appear to have based on your bone marrow biopsy.. Secondary (AA) amyloidosis is a reactive process caused by circulating serum amyloid A protein, and is often related to an underlying autoimmune disease, which you have as well. Thus you have two potential sources for your amyloidosis, and it's important to figure out which one is causing the deposits, since the treatments are very different: i.e. myeloma-like therapy to kill off the plasma cells if it's AL, and anti-inflammatory therapy to treat the autoimmune disease if it's AA.

In difficult cases like this, it's often helpful to be evaluated at a myeloma/amyloidosis specialty center, where additional testing can be done. In addition, the biopsy specimen showing the amyloid (in your case the rectal and bone marrow biopsies) can be sent to Mayo Clinic Laboratories, where they can do testing called mass spectrometry to definitvely determine which type of amyloid deposits you have. It may be worth discussing this with your oncologist to see if this testing can be ordered. Hope this is helpful.

[AS_AM]

Re: Significance of MGUS

by AS_AM on Thu Oct 03, 2013 2:20 pm

Thank You very much, Doctor Adam.
I have few more questions.
It is possible that one person can have both types of Amyloidosis AL and AA?
My next visit to hemato-oncologist will be very soon. What additional questions I have to ask him?
Thanks Igor,