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How do you approach the workup and initial treatment of systemic AL amyloidosis?

Once a tissue biopsy has confirmed light chain amyloid, what additional tests do you perform as part of a standard workup? In whom should treatment be initiated at diagnosis? Is systemic therapy always the preference, or can local modalities (eg. RT) be considered?
1 Answer
Mednet MemberInvited Expert
Medical Oncology · OhioHealth
Answered on

1: Ensure that this is light chain. Mass spectroscopy of the tissue to identify/confirm is very important. Even if a patient has positive serum monoclonal protein or light chain, it does not indicate that this is light chain amyloidosis. I have had patients with MGUS and smoldering myeloma with ATTR (transthyretin) amyloidosis and fibrinogen amyloidosis (approx 13% of patients with monoclonal gammopathy).

2: Do the routine labs as you would for multiple myeloma: serum free light chains, serum immunoglobulins, 24 hr urine total protein with immunofixation, bone marrow biopsy with cytogenetics/FISH, skeletal survey, albumin, beta-2-microglobulin, CBC, CMP, LDK, alk phos.

3: additional labs: troponin, BNP, NT proBNP, lipids (triglycerides can ...

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