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Amyloidosis: prognosis and treatment
1Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, MN 55905.
Seminars in Arthritis and Rheumatism
|October 1, 1994
Summary
Prognostic factors for amyloidosis survival include heart failure and neuropathy. While treatments like chemotherapy offer hope, new alternatives are needed for better patient outcomes in systemic amyloidosis.
Area of Science:
- Hematology
- Nephrology
- Rheumatology
Background:
- Amyloidosis is a group of plasma cell disorders characterized by extracellular deposition of misfolded proteins.
- Localized and systemic forms of amyloidosis present unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To review prognostic factors influencing clinical outcome and survival in amyloidosis.
- To evaluate current therapeutic strategies for localized and systemic amyloidosis.
Main Methods:
- Retrospective literature review of studies on amyloidosis treatment and prognosis since 1975.
- Analysis of prognostic indicators including congestive heart failure, beta 2-microglobulin, and neuropathy.
Main Results:
- Key adverse prognostic factors include monoclonal light chains, multiple myeloma, and hepatic involvement.
- Colchicine, chlorambucil, and dimethyl sulfoxide show efficacy in specific amyloidosis subtypes.
- Chemotherapy, dialysis, and transplantation represent advances in amyloidosis management, with some long-term survival reported.
Conclusions:
- Despite treatment advances, median survival for primary systemic amyloidosis remains poor (2.1 years).
- Further research is crucial to develop more effective therapies and improve long-term survival for amyloidosis patients.