How is light chain amyloidosis (AL) diagnosed and treated?

Updated: May 09, 2019
  • Author: Robert O Holmes, Jr, DO; Chief Editor: Herbert S Diamond, MD  more...
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The precursor protein is a clonal immunoglobulin light chain or light chain fragment. AL is a monoclonal plasma cell disorder closely related to multiple myeloma, as some patients fulfill diagnostic criteria for multiple myeloma. Typical organs involved include the heart, kidney, peripheral nervous system, gastrointestinal tract, respiratory tract, and nearly any other organ. AL includes former designations of primary amyloidosis and myeloma-associated amyloidosis.

Treatment usually mirrors the management of multiple myeloma (ie, chemotherapy). Selected patients have received benefit from high-dose melphalan and autologous stem-cell transplantation, with reports of prolonged survival in some studies.

The most current guidelines recommend high-dose steroids for isolated organ involvement, but transplantation should be considered early. [36] Any transplantation should also be followed by high-dose intravenous melphalan supported with stem-cell transplantation to try to prevent future amyloid deposition in the transplanted organ. [36]

Other agents used in AL amyloidosis have included bortezomib, rituximab, immunomodulatory agents, and standard-dose alkylating agents (eg, melphalan, cyclophosphamide), thalidomide, and lenalidomide. [37, 38, 36, 39] Bortezomib is a proteasome inhibitor that is well tolerated in multiple myeloma. [40, 41] Patients younger than 65 years may be candidates for stem cell transplantation with melphalan and dexamethasone or thalidomide-cyclophosphamide-dexamethasone regimens. [36]

The response is then classified as either partial, complete, or no response. If a partial or complete response is achieved, patients are closely monitored. [36] Imaging and some biomarkers like N-terminal pro-brain natriuretic peptide (NT-proBNP), B-type natriuretic peptide (BNP), troponin, and free light-chain concentration can be useful in gauging clinical response, especially in cardiac amyloid disease. [39] If no response is seen, the patient becomes a candidate for novel agents that usually include alkylating agents combined with novel agents such as lenalidomide. [36, 42]

For more information, see Immunoglobulin-Related Amyloidosis.

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