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NCCN Flash Update: NCCN Guidelines Updated for Systemic Light Chain Amyloidosis

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines^®), the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™) for Systemic Light Chain Amyloidosis. These NCCN Guidelines^® are currently available as Version 1.2026.

Link directly to the Updates section of the NCCN Guidelines: Systemic Light Chain Amyloidosis

AMYL-1

• Clinical and amyloid-related assessment, bullet 6 added: 6-minute walk test.
• Footnote c added: The 6-minute walk test is used to assess aerobic capacity and endurance and serves as a predictor of cardiac function. Cohen OC, et al. Heart 2022;108:1616-1622.
• Footnote d modified: It is essential to confirm that patients have primary SLCA rather than hereditary amyloidosis, wild-type transthyretin-related (amyloid transthyretin [ATTR]) cardiac amyloidosis, or secondary amyloidosis. The amyloid deposits should be confirmed to be composed of light chains using immunohistochemistry or MS. Immunohistochemistry for transthyretin or serum amyloid A component should be performed if kappa and lambda stains are negative. In the absence of monoclonal protein or elevated light chains, 99mTc-pyrophosphate (PYP) scan can help diagnose distinguish cardiac involvement with SLCA from ATTR.
• Footnote e modified: Identification of light chains in the serum or urine without confirmation of the amyloid deposition composition in tissue is not adequate, as patients with other forms of amyloidosis may have an unrelated monoclonal gammopathy of undetermined significance (MGUS). Lachmann HJ, et al. N Engl J Med 2002;346:1786-1791.

AMYL-4

• Column 4, clinical finding added: ...protein or elevated FLC with abnormal ratio present.
• Footnote v added: Previous tissue biopsy (within last 1-2 years) can be stained with Congo red.

AMYL-B (1 of 6)

• Bullet 3 modified: Modification of treatment should be considered Iif hematologic (biochemical) responseis< very good partial response (VGPR) by cycle 3 or < partial response (PR) by cycle 2 of initial therapy, or andno complete response (CR) and persistent organ dysfunction. eventually organ response, consider treatment modification.
• Bullet 4 added: Typically, light-chain normalization precedes organ response.
• Sub-header added: Supportive Care.
• Supportive Care, bullet added: There are several supportive care measures that should be considered. See Table 1 in Maroun BZ, et al. Blood Res 2022;57:106-116.

AMYL-B (2 of 6)

• Significant neuropathy, All stages, Melphalan/Dexamethasone (if ineligible for HCT) moved from Preferred to Useful in Certain Circumstances.
• Footnote c added: Patients with IgM paraprotein may require different therapy. If there is underlying plasma cell clone, follow suggested therapies. For lymphoma or LPL, see NCCN Guidelines for Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma.

AMYL-B (4 of 6)

• Useful in Certain Circumstances, regimen added: Venetoclax t(11;14) + Daratumumab.

AMYL-B (6 of 6)

• Reference added for Venetoclax/Daratumumab.

NCCN has published updates to the NCCN Chemotherapy Order Templates (NCCN Templates^®) for Systemic Light Chain Amyloidosis to reflect the currently published NCCN Guidelines for Systemic Light Chain Amyloidosis v1.2026.

• The following New NCCN Templates^® have published:
  • SLCA24: Venetoclax/DexAMETHasone

• Changes to the Indication section have been made on the following templates:
  • SLCA26: Dose-Modified Bortezomib/CycloPHOSphamide/DexAMETHasone

• Regimen names may have been updated in this library to reflect NCCN standard naming conventions
• Drug information notes for the following agents have been updated in the Supportive Care, Monitoring and Hold Parameters, and/or Safety Parameters and Special Instructions sections:
  •  Daratumumab
  • Daratumumab and hyaluronidase-fihj
  • Ixazomib

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Biomarkers Compendium^®, the NCCN Chemotherapy Order Templates (NCCN Templates^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

To view the NCCN Guidelines for Patients^®, please visit NCCN.org/patientguidelines.

Free NCCN Guidelines apps for iPhone, iPad, and Android devices are now available! Visit NCCN.org/apps.

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