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Primary amyloidosis (AL) 

Primary amyloidosis (AL)
Chapter:
Primary amyloidosis (AL)
Author(s):

Robert A. Kyle

DOI:
10.1093/med/9780199579655.003.0113
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date: 08 April 2020

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.

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