Chapter 39 Immunoglobulin Light-Chain Amyloidosis (Primary Amyloidosis)
Table 39-1 Nomenclature of Amyloidosis
ABPP, Amyloid β protein precursor; GI, gastrointestinal; SAA, serum amyloid A.
From Gertz MA, Lacy MQ, Dispenzieri A: Immunoglobulin light chain amyloidosis (primary amyloidosis, AL). In Gertz MA, Greipp PR, editors: Hematologic malignancies: Multiple myeloma and related plasma cell disorders. New York, 2004, Springer-Verlag, p 157. Used with permission of Mayo Foundation for Medical Education and Research.
Table 39-2 Syndromes in Primary Amyloidosis
| Syndrome | Patients (%) |
|---|---|
| Nephrotic or nephrotic and renal failure | 30 |
| Hepatomegaly | 24 |
| Congestive heart failure | 22 |
| Carpal tunnel | 21 |
| Neuropathy | 17 |
| Orthostatic hypotension | 12 |
From Gertz MA, Lacy MQ, Dispenzieri A: Amyloidosis. In Mehta J, Singhal S, editors: Myeloma. London, 2002, Martin Dunitz Ltd., p 445. Used with permission.
Figure 39-1 A, Bone marrow biopsy specimen showing thickened large and small vessels caused by amyloid deposition. B, Rectal biopsy specimen demonstrates amyloid deposits (left) with Brunner glands (right). (Congo red; original magnification, ×100.)
Diagnostic Pathway for Primary Amyloidosis
Consider primary amyloidosis in patients with
Confirm diagnosis histologically
Melphalan–dexamethasone–lenalidomide
Cyclophosphamide–thalidomide–dexamethasone
Cyclophosphamide–bortezomib–dexamethasone
NT-pro-BNP, N-terminal pro-brain natriuretic peptide.
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