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Immunoglobulin light chain amyloidosis

Guidelines

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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Screening and diagnosis

Clinical presentation: as per BCSH 2015 guidelines, 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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Diagnostic investigations

Laboratory tests: as per BCSH 2015 guidelines, 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

Diagnostic procedures

Biopsy and pathology: as per BCSH 2015 guidelines, perform biopsy of an apparently affected organ, if possible, for histological diagnosis in patients with suspected AL amyloidosis.
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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

Nonpharmacologic interventions

Nutrition
As per BCSH 2015 guidelines:
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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Therapeutic procedures

ASCT: 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

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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Specific circumstances

Patients with IgM-related amyloidosis
As per EHA/ISA 2023 guidelines:
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

Patient education

General counseling: as per BCSH 2015 guidelines, provide appropriate information and support in patients, their families and carers as an essential component of management.
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Preventative measures

Thromboprophylaxis
As per BCSH 2015 guidelines:
Consider administering prophylactic LMWH in patients at high risk of VTE.
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Consider administering aspirin in patients at low risk of VTE.
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  • Prophylactic antibiotics

Follow-up and surveillance

Monitoring of treatment response: as per BCSH 2015 guidelines, 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