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NCCN Flash Updates: 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 1.2024.

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

AMYL-1

Laboratory evaluation (directed toward commonly affected organ systems):
- 9th bullet modified:NT-proBNP/BNP troponin T (TnT)
Pathologic evaluation
- 5th bullet added:If lymphoplasmacytic clone is present, then test for MYD88 L265P mutation.
Special testing based on organ system involvement: section moved to AMYL-3
Pathway added:link to clinical suspicion for cardiac amyloidosis (AMYL-3)
Pathway added:link to clinical suspicion for localized amyloidosis (AMYL-4)
Special testing based on organ system involvement pathway moved to (AMYL-2)

10th bullet modified: Liver function tests (LFTs): alkaline phosphatase (ALP), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and bilirubin

12th bullet added: Lipid panel

AMYL-2

Special testing based on organ systems moved from (AMYL-1) to (AMYL-2) as part of the diagnostic workup

AMYL-3

New page: Clinical suspicion for cardiac amyloidosis

AMYL-4

New page: Clinical suspicion for localized amyloidosis

AMYL-A 1 of 6

General considerations:
- 1st bullet added:Goal of treatment is to achieve a complete hematologic response
Screening and Prophylaxis Recommendations
- 3rd bullet added: Recommend herpes zoster prophylaxis for patients treated with proteasome inhibitors or daratumumab
Side Effects and Lab Tests

3rd bullet added:Patients achieving < VGPR by cycle 3 or < PR by cycle 2 of primary therapy should be considered for treatment modification.

4th bullet added:Amyloid-targeting agents (eg, doxycycline or anti-febrile antibodies) are not recommended outside clinical trials.

5th bullet added: For systemic light chain amyloidosis (SLCA) with an underlying lymphoplasmacytic clone, treat underlying lymphoplasmacytic lymphoma/Waldenström macroglobulinemia as outlined in the NCCN Guidelines for Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma

3rd bullet modified:Treatment with immunomodulatory drugs is associated with transient elevation of cardiac biomarkers. Patients with cardiac amyloid should be carefully monitored while on ~~lenalidomide~~ therapy with immunomodulatory drugs.

AMYL-A 2 of 6

Page extensively modified: Primary treatment for HCT-eligible and non-eligible patients with SLCA table re-organized based on patient characteristics and Mayo 2004 staging.

AMYL-A 3 of 6

New page: Treatment considerations for newly diagnosed SLCA

added: considerations for autologous HCT

added: definite exclusions for autologous HCT

AMYL-A 4 of 6

Therapy for previously treated disease:

Regimen added:Ixazomib/cyclophosphamide/dexamethasone

AMYL-A 5 of 6

References added

AMYL-A 6 of 6

References added

AMYL-B

Reference added
Footnote h added:Characteristic findings on cardiac MRI: global subendocardial late gadolinium enhancement (subendocardial or transmural involvement) with abnormal myocardial and blood-pool gadolinium kinetics. Note: This is not part of the consensus criteria.

AMYL-C

Staging systems were updated with Mayo 2012 Staging System and Mayo 2004 Staging System with European Modifications

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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