NCCN Clinical Practice Guidelines In Oncology (NCCN Guidelines®)

Ibrutinib (IMBRUVICA®) ± rituximab: state NCCN Guidelines® Category 1 preferred option in both frontline and previously-treated WM1

Waldenström Macroglobulinemia therapy (order of regimens is alphabetical by category)1

Note: All recommendations are Category 2A unless otherwise indicated.

Preferred Regimens

  • Bendamustine/rituximab
  • Bortezomib/dexamethasone/rituximab
  • Ibrutinib ± rituximab (category 1)
  • Zanubrutinib (category 1)

Other Recommended Regimens

  • Bendamustine
  • Carfilzomib/rituximab/dexamethasone
  • Ixazomib/rituximab/dexamethasone
  • Rituximab
  • Rituximab/cyclophosphamide/dexamethasone
  • Rituximab/cyclophosphamide//prednisone

Preferred Regimens

  • Ibrutinib ± rituximab (category 1)
  • Zanubrutinib (category 1)
  • Bendamustine/rituximab
  • Bortezomib/dexamethasone/rituximab
  • Rituximab/cyclophosphamide/dexamethasone

Other Recommended Regimens

  • Acalabrutinib
  • Bendamustine
  • Cyclophosphamide/doxorubicin/vincristine/prednisone/rituximab
  • Ixazomib/rituximab/dexamethasone
  • Rituximab
  • Rituximab/cyclophosphamide/prednisone
  • Venetoclax

Useful In Certain Circumstances

  • Everolimus
  • Ofatumumab (for rituximab-intolerant individuals)
  • Cladribine ± rituximab
  • Fludarabine ± rituximab
  • Fludarabine/cyclophosphamide/rituximab

Hematopoietic Cell Transplant

  • In selected cases hematopoietic cell transplantation may be appropriate with either:
    • Autologous hematopoietic cell transplant
    • Allogeneic hematopoietic cell transplant (ablative or nonablative)

Primary therapy

Preferred Regimens

  • Bendamustine/rituximab
  • Bortezomib/dexamethasone/rituximab
  • Ibrutinib ± rituximab (category 1)
  • Zanubrutinib (category 1)

Other Recommended Regimens

  • Bendamustine
  • Carfilzomib/rituximab/dexamethasone
  • Ixazomib/rituximab/dexamethasone
  • Rituximab
  • Rituximab/cyclophosphamide/dexamethasone
  • Rituximab/cyclophosphamide//prednisone

Previously treated WM

Preferred Regimens

  • Ibrutinib ± rituximab (category 1)
  • Zanubrutinib (category 1)
  • Bendamustine/rituximab
  • Bortezomib/dexamethasone/rituximab
  • Rituximab/cyclophosphamide/dexamethasone

Other Recommended Regimens

  • Acalabrutinib
  • Bendamustine
  • Cyclophosphamide/doxorubicin/vincristine/prednisone/rituximab
  • Ixazomib/rituximab/dexamethasone
  • Rituximab
  • Rituximab/cyclophosphamide/prednisone
  • Venetoclax

Useful In Certain Circumstances

  • Everolimus
  • Ofatumumab (for rituximab-intolerant individuals)
  • Cladribine ± rituximab
  • Fludarabine ± rituximab
  • Fludarabine/cyclophosphamide/rituximab

Hematopoietic Cell Transplant

  • In selected cases hematopoietic cell transplantation may be appropriate with either:
    • Autologous hematopoietic cell transplant
    • Allogeneic hematopoietic cell transplant (ablative or nonablative)

NCCN Categories of Evidence and Consensus1

Category 1: Based upon high-level evidence, there is uniform NCCN consensus that the intervention is appropriate.

Category 2A: Based upon lower-level evidence, there is uniform NCCN consensus that the intervention is appropriate.​
 

NCCN Categories of Preference1

Preferred intervention: Interventions that are based on superior efficacy, safety, and evidence; and, when appropriate, affordability.

Other recommended intervention: Other interventions that may be somewhat less efficacious, more toxic, or based on less mature data; or significantly less affordable for similar outcomes.

Useful in certain circumstances: Other interventions that may be used for selected patient populations (defined with recommendation).

All recommendations are considered appropriate.

NCCN Guidelines

Access the NCCN Guidelines for Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma.

Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. 

Adapted with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma V.1.2023. © National Comprehensive Cancer Network, Inc. 2022. All rights reserved. The NCCN Guidelines® and illustrations herein may not be reproduced in any form for any purpose without the express written permission of the NCCN. To view the most recent and complete version of the NCCN Guidelines, go online to NCCN.org. The NCCN Guidelines® are a work in progress that may be refined as often as new significant data becomes available. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.

Reference: 1Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma V.1.2023. © National Comprehensive Cancer Network, Inc. 2022. All rights reserved. Accessed March 1, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.