Abstract:

Primary systemic immunoglobulin light chain amyloidosis (AL) results from the accumulation of amyloid fibrils that are composed of a monoclonal immunoglobulin light chain that is produced by a plasma cell clone. Almost every organ can be involved, resulting in multisystemic symptoms and signs. Diagnosis may be difficult and requires a certain degree of suspicion. Treatment is challenging and most patients may be quite frail. Careful assessment of the organ involvement and prognosis is needed before planning the treatment strategy. Supportive care is critical for AL patients. The goal of treatment is the reduction of light chain production. Conventional chemotherapy may be effective and recently introduced novel agents expand treatment options in patients who relapse or are not candidates for high-dose melphalan with autologous stem cell transplant (HDM-ASCT). In experienced centers, HDM-ASCT in patients deemed capable of tolerating the procedure is associated with high response rates with modest treatment-related mortality. © 2011 Blackwell Publishing Ltd.

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Export Date: 21 February 2017

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