Al Amyloidosis
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Contents |
Etiology
- overproduction of monoclonal light chains (80% lambda)
- a localized amyloidosis involving a single organ with deposition of light chains may occur
Pathology
- fibrils contain NH2 terminal amino acid residues of the variable end of the light chain
- increased risk of hemorrhage
- systemic fibrinolysis
- acquired factor X deficiency
- increased vascular fragility
Clinical-manifestations
- nephrotic syndrome (28%)
- carpal tunnel syndrome (21%)
- congestive heart failure (17%)
- peripheral neuropathy (17%)
- orthostatic hypotension (11%)
- hepatomegaly
- malabsorption
Laboratory
-
- elevated serum alkaline phosphatase
- 1st indicator of liver involvement
- albuminuria
- may show free light chains
- serum protein electrophoresis is generally normal
- immunofixation electrophoresis (IFE) may show free light chains
Management
- median survival is 1 year (overall)
- < 3-4 months with CHF & LVEF < 40%
- nephrotic syndrome alone: 2.5 years
- peripheral neuropathy alone: 5 years
- chemotherapy is unsatisfactory
- low plasma cell proliferation rate
- prednisone + melphalan may be of some benefit, especially in patients with nephrotic syndrome but without renal insufficiency or cardiac involvement
- high-dose melphalan with stem cell transplantation may benefit a subset of patients
More General Terms
Additional Terms
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 15 American College of Physicians, Philadelphia 1998, 2009
- National Guideline Clearinghouse Guidelines on the diagnosis and management of AL amyloidosis. British Committee for Standards in Haematology ngc-guideline: [1]
