Chapter

Primary amyloidosis (AL)

Robert A. Kyle

in Rheumatology and the Kidney

Second edition

Published on behalf of Oxford University Press

Published in print April 2012 | ISBN: 9780199579655
Published online February 2013 | e-ISBN: 9780191763472 | DOI: http://dx.doi.org/10.1093/med/9780199579655.003.0113

Series: Oxford Clinical Nephrology Series

Primary amyloidosis (AL)

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1. Must determine the type of amyloid protein by immunohistochemistry or mass spectrometry. 2. A monoclonal (M) protein is present in serum in 70% of patients at diagnosis and in almost 90% if both serum and urine are evaluated. 3. Nephrotic syndrome or renal failure is the presenting feature in 30% of AL patients. 4. Other major features at presentation are congestive heart failure (20%), carpal tunnel syndrome (20%), peripheral neuropathy (15%) or orthostatic hypotension (10%). 5. Biopsy of subcutaneous fat and bone marrow are positive in almost 90% of AL patients. 6. Autologous stem cell transplantation is feasible in approximately 20%. Cardiac involvement manifested by troponin T ≥0.06 mg/ml, performance status ≤2 or significant involvement of >2 organs are contraindications for autologous transplantation. 7. Major chemotherapy regimens include melphalan and dexamethasone or bortezomib and dexamethasone. 8. A useful prognostic model utilizes NT-proBNP >332 mg/l, troponin T >0.035 µg/l and troponin I >0.10 µg/l.

Chapter.  3864 words.  Illustrated.

Subjects: Nephrology

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