Canterbury DHB

Context

Amyloidosis

10% of patients with myeloma will have evidence of amyloidosis. Most patients presenting with primary amyloidosis do not have myeloma, at least at presentation, but will have a monoclonal protein. For a recent review article on diagnosis and prognosis, see Gertz, ASH EPB, 2004.

Amyloid is an infiltrative, fibrillary protein with light chain sequences identical to those of the light chains produced by the monoclonal plasma cells.

Suspect amyloid in a patient with a monoclonal protein who presents with:

In This Section

Diagnosis

Management

Diagnosis

Apple green bi-refringence on polarised light examination of a tissue biopsy stained by Congo Red establishes the diagnosis. Subcutaneous abdominal fat biopsy is minimally invasive and can sometimes be used to confirm the diagnosis. Otherwise the affected organ should be biopsied.

Survival is usually poor and such patients have proven difficult to treat. Some patients benefit from standard anti-myeloma therapy, but most do not. Improved results have been seen following stem cell autografts and confirmation of these results is awaited.

Management

An approach similar to that used in myeloma is often used. CyBorD and C-Thal-Dex are tolerable and show efficacy. Autologous stem cell transplant remains controversial in amyloid and may even decease survival.

For current trials in Amyloidosis, see the Clinical Trials page on the Intranet.

About this Canterbury DHB document (5614):

Document Owner:

Sean Macpherson (see Who's Who)

Issue Date:

September 2014

Next Review:

September 2016

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 5614