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Immunoglobulin light chain amyloidosis

Key sources
The following summarized guidelines for the evaluation and management of immunoglobulin light chain amyloidosis are prepared by our editorial team based on guidelines from the International Society of Amyloidosis (ISA/EHA 2023; 2022), the American College of Radiology (ACR 2021), the Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART 2021), and the British Committee for Standards In Haematology (BCSH 2015).
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Guidelines

1.Screening and diagnosis

Clinical presentation: recognize that AL amyloidosis is a disease with insidious onset and considerable clinical heterogeneity and high level of clinical suspicion is essential to avoid delayed diagnosis.
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2.Diagnostic investigations

Laboratory tests: obtain comprehensive assessment of the extent of organ involvement and dysfunction using noninvasive criteria, including obtaining serum amyloid P component scanning when feasible.
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  • Cardiac imaging

  • Nerve conduction studies

3.Diagnostic procedures

Biopsy and pathology: perform biopsy of an apparently affected organ, if possible, for histological diagnosis in patients with suspected AL amyloidosis.
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4.Medical management

General principles: as per mSMART 2021 guidelines, initiate treatment immediately in virtually all patients with systemic AL amyloidosis.
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  • Goals of treatment

  • First-line regimens

  • Management of cardiac involvement (chemotherapy)

  • Management of renal involvement

  • Management of peripheral nerve involvement

  • Management of autonomic dysfunction

  • Management of bleeding

  • Management of cardiac involvement (supportive therapy)

  • Maintenance and consolidation therapy

5.Nonpharmacologic interventions

Nutrition
Offer nutritional supplementation in patients with gastrointestinal symptoms.
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Consider administering TPN if gastrointestinal symptoms are disabling, and the patient becomes malnourished.
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6.Therapeutic procedures

Autologous stem cell transplantation: as per mSMART 2021 guidelines, consider administering high-dose chemotherapy with ASCT in selected patients with AL amyloidosis.
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  • Allogenic stem cell transplantation

7.Surgical interventions

Solid organ transplantation: as per mSMART 2021 guidelines, consider performing heart transplantation in very selected patients with AL amyloidosis.
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8.Specific circumstances

Patients with immunoglobulin M-related amyloidosis
Offer rituximab and bendamustine or ASCT as first-line therapy in patients with IgM-related amyloidosis.
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Offer the following regimens as alternative therapies in patients with IgM-related amyloidosis:
rituximab, bortezomib, and dexamethasone
rituximab, cyclophosphamide, and dexamethasone
cyclophosphamide, bortezomib, dexamethasone
ibrutinib with or without rituximab
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  • Patients with t(11;14) translocation

  • Patients with localized amyloidosis

9.Patient education

General counseling: provide appropriate information and support in patients, their families and carers as an essential component of management.
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10.Preventative measures

Thromboprophylaxis
Consider administering prophylactic LMWH in patients high risk of VTE.
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Consider administering aspirin in patients at low risk of VTE.
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  • Prophylactic antibiotics

11.Follow-up and surveillance

Monitoring of treatment response: obtain free light chains or M-protein for monitoring of treatment response after each cycle of chemotherapy during treatment and every 1-3 months thereafter.
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  • Management of relapsed/refractory disease