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.
Original language | English (US) |
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Pages (from-to) | 156-164 |
Number of pages | 9 |
Journal | Amyloid |
Volume | 29 |
Issue number | 3 |
DOIs | |
State | Published - 2022 |
Keywords
- ATTR
- Bone marrow
- amyloidosis
- cardiac involvement
- proteomics
- typing
ASJC Scopus subject areas
- Internal Medicine