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Abstract Details

AL Versus TTR Amyloidosis and Their Relationship to Inflammatory Infiltrates in Nerve
Peripheral Nerve
P05 - (-)
069
BACKGROUND: The association of amyloid and inflammation in nerve biopsies has not been systematically studied. Such an association might be suspected, since amyloidogenesis is known to be promoted by the inflammatory milieu, and since primary amyloidosis (AL) occurs in the context of immune cell dyscrasia.
DESIGN/METHODS: All biopsies in the Mayo Clinic Peripheral Nerve Laboratory database diagnostic of amyloidosis between 1995 and 2012 were identified. Biopsies were graded for presence, size, and location of inflammatory collections. Medical records were reviewed for amyloid subtype, and clinical and demographic features.
RESULTS: Of 125 biopsies reported to be diagnostic of nerve amyloidosis, 104 were available for review. 90 biopsies had defined subtype: 48 AL, 40 transthyretin (TTR), and 2 gelsolin. 29 AL cases had immunoglobulin subtyping: 2 IgA, 17 IgG, and 10 IgM. 26 TTR cases had specific mutations identified, with a Val30Met mutation in 12. Amyloid was seen most commonly in the endoneurium, usually in close approximation to endoneurial blood vessels. Abnormal inflammation was seen in 52/104 biopsies, with moderate to large collections seen in 20. A significantly larger proportion of AL cases demonstrated moderate/large inflammatory collections than TTR cases. Inflammation was seen more often in IgM amyloid subtype than IgG and IgA.
CONCLUSIONS: Abnormal inflammation was seen in biopsies diagnostic of amyloidosis in half of cases. AL amyloid (due to immune dysregulation) had significantly more inflammation than did TTR amyloid (due to genetic defect). IgM amyloid was more associated with abnormal inflammation than other subtypes. While these results are preliminary, they point to an underlying dysregulation of the immune system in AL amyloid neuropathy.
Authors/Disclosures
Adam Loavenbruck, MD (University of Minnesota)
PRESENTER 		Dr. Loavenbruck has nothing to disclose.
Peter J. Dyck, MD, FAAN (Mayo Clinic) Dr. Dyck has nothing to disclose.
Wolfgang Singer, MD, FAAN (Mayo Clinic) Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. The institution of Dr. Singer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Lundbeck. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ionis. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Yoda. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Theravance. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ferrer. The institution of Dr. Singer has received research support from NIH. The institution of Dr. Singer has received research support from FDA. The institution of Dr. Singer has received research support from Michael J. Fox Foundation. Dr. Singer has received intellectual property interests from a discovery or technology relating to health care.
Gilles Edan Gilles Edan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Celgene. Gilles Edan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Gilles Edan has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Biogen. Gilles Edan has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Merck.
P. James B. Dyck, MD, FAAN (Mayo Clinic) Dr. Dyck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Akcea/Ionis.