[jellylants]

AL Amyloidosis Myeloma

by jellylants on Sun Feb 10, 2013 10:09 am

Chicken or egg?? what comes first, Myeloma or Amyloid ??
I lost my husband of 39 years in November 2011 to AL Amyloidosis/Myeloma, a fit and healthy golfer/fisherman until he began to get shortness of breath in 2010, tiredness, lethargy etc.,
His diagnosis sadly took too long and by the time he began low chemo it was too late. His heart was the organ affected by the amyloid, albeit the chemo did reduce the light chains dramatically in only 3 weeks he had come down from 264 to only 19 !!
We got a bit excited at that stage as he was ony 63, thought he might just make it for a few more years.
He could not taste anything apart from grapefruit and acidic fizzy drinks. He would drule over the cooking channels on tv as he had an appetite but could not physically take anything textured. In the end a month later he died literally of thirst and hunger, the worst possible way to die in my opinion.
My concern today (Sunday 10th Feb 2013) his older brother 66yrs is in as I write undergoing an operation at Guys hospital to remove fluid from the lungs and inject with a powder like substance, he has the same symptoms.
We were told that Den's amyloid type was non hereditory, so how come his healthy brother has the same symptoms, ie breathless, tired, weight loss etc., I do not believe in coincedences, surely this cannot be true??

[Dr. Ken Shain]

Re: AL Amyloidosis Myeloma

by Dr. Ken Shain on Tue Feb 12, 2013 11:11 am

I am very sorry to hear about your husband and now the difficulties with your brother-in-law. We wish only the very best for your brother-in-law.

Amyloidosis is quite a complicated number of diseases. There are over a dozen different causes of amyloidosis. Only 1 of which is primary amyloidosis- amyloid causes by misfoled immunoglobulin light chains. Primary amyloidosis (AL) (a kin to myeloma) is a"non-hereditary." There are specific hereditary types for amyoidosis including, but not limited to ATTR (transthyretinin), AFib (Fibrinogen alpha), AAPoAI (apolipoprotein AI) and others. Therse have well established modes of inheritance.

Primary AL, like multiple myeloma, is not generally an inherited disorder meaning that there are not specific inheritance patterns (Of note, 10-15% of multiple myeloma patients will also have light chain amyloidosis). These are both "spontaneous" malignancies. Importantly, this is not to say that myeloma and/or primary AL cannot be seen in families- as in your case. We all have patients that have multiple family members with these disease or like B cell disorders. This is very rare and unfortunately we do not yet know how to predict these events or what causes them. Hopefully, the growing technology and quality of genomic analysis will aide in identifying specific genes or loci.

Of utmost importance in any case of AL is to correctly identify the type of amyloidosis, because the treatments for each will be significantly different. I always recommed finding a center of excellence for evaluation. There are a number of tests that can be utilized to determine the origin of the amyloid protein.

If identifyed as primary AL or associated with active multiple myeloma, then the treatments are focused on controlling the malignant plasma cell population causing the disase. Unfortunately, cardiac involvement is a very significant barrier to certain modes of therapy (HDM-ASCT) and a significant risk factor for long term survival.