Bone Marrow Amyloid: A Comprehensive Analysis of 1,469 Samples, Including Amyloid Type, Clinical Features, and Morphologic Distribution | oneAMYLOIDOSISvoice
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Bone Marrow Amyloid: A Comprehensive Analysis of 1,469 Samples, Including Amyloid Type, Clinical Features, and Morphologic Distribution

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source: Amyloid: The Journal of Protein Folding Disorders

year: 2022

authors: April Chiu, Surendra Dasari, Paul J. Kurtin, Jason D. Theis, Julie A. Vrana, Angela Dispenzieri, Karen L. Rech, Linda N. Dao, Matthew T. Howard, Martha Grogan, Ellen D. McPhail

summary/abstract:

Background: Bone marrow biopsy is common in patients suspected of having systemic AL amyloidosis. However, little is known about the incidence, morphology and clinical phenotype of non-AL amyloid types in bone marrow.
 
Methods: We retrospectively identified N = 1469 bone marrow amyloid biopsies typed using a proteomics-based method between 2008-2020. Frequency of amyloid types (N = 1469), distribution of amyloid deposits (N = 139), and clinical phenotypes (N = 355), with particular emphasis on cardiac involvement, were assessed.
 
Results: The amyloid types were: AL (N = 1172; 79.8%), ATTR (N = 240; 16.3%), AH (N = 38; 2.6%), AA (N = 17; 1.2%), and Aβ2M (N = 2; 0.1%). Although there were characteristic morphologic features, including periosteal soft tissue and/or vascular involvement in ATTR, interstitial vascular involvement in AA, and variable anatomic compartment involvement in AL, none were pathognomonic. Most patients with both an M-spike and cardiac involvement had AL amyloid in their BM, but in over 10% the amyloid type was ATTR. Compared to AL patients, ATTR patients had higher stage cardiac amyloidosis and lower overall survival, which was mainly due to advanced cardiac stage.
 
Conclusions: ATTR amyloid is common in bone marrow and its morphologic distribution overlaps with AL. Amyloid typing is critical as over 10% of patients with bone marrow amyloid, cardiac amyloidosis, and an M-spike have ATTR amyloidosis.
 
organization: Mayo Clinic, USA

DOI: 10.1080/13506129.2022.2031963

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