Amyloidosis Flashcards
cornerstone of treatment
proteosome inhibitors
standard of care if transplant ineligible for AL amyloidosis
Dara-Cy-Bor-D
Coagulopathy associated with light chain amyloidosis
- acquired deficiency of factor x (occurs via absorption of factor X onto amyloid fibrils)
diagnosis
- tissue biopsy of involved organ OR abdominal fat pad OR via bone marrow aspiration (via IHC staining)
- amyloid deposition can be seen by Congo red staining or electron microscopy
types of amyloid
1) Systemic light chain
2) wild-type transthyretin-related cardiac
3) secondary
4) hereditary amyloidosis
management of hereditary amylodiosis
liver transplant (amyloidogenic protein is synthesized solely by the liver)
How to definitively rule out amyloid
- must use electron microscopy, IHC, or mass spectrometry to determine amyloid deposits are composed of light chains. Identification of light chains in serum or urine without confirmation of amyloid composition is not adequate.
What is the relationship between AL amyloidosis and multiple myeloma?
- main problem in AL amyloidosis is buildup of light chains that fold abnormally and produced by abnormal cells
- there can be overlap between the two disease and patients are frequently diagnosed with both
Basic pathophysiologic problem with amyloidosis
It is a protein misfolding disease
Transplant eligibility criteria
1) Age <70
2) SBP >90
3) NYHA <3
4) No more than 2 organs involved
5) Troponin T level <0.06
6) NTproBNP <5k
7) ECOG 0-1
Management of transplant eligible AL amyloid patient with high bone marrow clonal plasma cell population
IF >10% bone marrow clonal plasma cells, 2-4 cycles of induction w/ bortezomib/cyclophosphamide/dexamethasone then auto-transplant
How is AL amyloidosis distinct from MM?
Both are characterized by overproduction of monoclonal light chains but in primary amyloidosis, patients develop tissue deposits of amyloid fibrils. AND they usually have few bone marrow plasma cells
