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4yr
Waldenstrom Macroglobulinemia patient with incomplete response – next steps?

50 year old male with unclear B cell lymphoma, watched for 3-4 years. When presented to care, had diffuse low volume adenopathy. Biopsy with molecular diagnostics was repeated, which showed Waldenstrom Macroglobulinemia (WM) based on a NMYD88 mutation. Patient was observed for 3-4 months with increasing peripheral edema. Patient was restaged and found minimal FDG avid nodes in systemic nodes, bone marrow with WM. Patient was started on RBendamustine (other options were ibrutinib vs others). Patient had incomplete response to RBenda. Renal biopsy was performed because of hypoalbumineia and renal amyloid was identified.

How would you have managed this patient and his treatment regimen(s)? What would you recommend as next steps?

  • 4yr
    I think a BTK inhibitor, such as Imbruvica or Calquence, would be reasonable.
  • 4yr
    What are your thoughts on BTK inhibitors?
  • 4yr
    1. goal of treatment would be important, treatment of underlying WM is important, which will help with renal amyloidosis.
    BTKi-preferably newer generation i.e zanubrutinib would be an option, and if progression of disease, consider chemotherapy based regimen such has CyBorDex +/- rituximab, consider autoSCT. Other options also include combination of venetoclax + ibrutinib or ven/Acala as off label, or single agent venetoclax after progression of disease on BTKi
  • 4yr
    I would start a BTKi, my preferred one is acalrubitinib.
  • 4yr
    tough case. Seems like a WM associated amyloid deposition- one can treat him with Velcade based therapy ( Velcade +dex+/- Rituxan) or Kyprolis +rituxan +dex or Ixazomib+RItuxan+Dex.; Alternatively Calquence / Ibrutinib or Zanubritinib are other options .
  • 4yr
    Would check myeloma markers as well and may need to direct therapy for myeloma accordingly
  • 4yr
    No standare of care but due to MYD88 would suggest use of BTK inhibitors perhaps acalabrutinib or zanubrutinib
  • 4yr
    No one standard of care, but given MYD88 mutation zanubrutinib is reasonable approach
  • 4yr
    I think zanubrutinib is a reasonable option given MYD88 mutation
  • 4yr
    BTK inhibitors seem to be effective in this patient population combined with Rituxan. I have used Ibrutinib but Acala and Zanu are likely better drugs with equal efficacy and better side effect profile. The consideration for a transplant certainly seems reasonable and should be arranged sooner than later
  • 4yr
    Since patient has MYD88 mutation I would recommend zanubrutinib.
  • 4yr
    Since patient has MYD88 mutation I would recommend zanubrutinib.
  • 4yr
    Since patient has MYD88 mutation I would recommend zanubrutinib.
  • 4yr
    i will treat with zanubtrutinib
  • 4yr
    It is well known that amyloid deposition is a possible complication of Waldenström macroglobulinemia associated with the immunoglobulin M protein. The treatment of WM has evolved rapidly, with treatment options that include anti-CD20 monoclonal antibody-based combinations and BTK inhibitors. The choice of therapy is based on the need for rapid disease control, presence of specific disease complications, and patient’s age. With the use of BTK inhibitors, the use of continuous therapy has been introduced as another option over fixed-duration chemoimmunotherapy. May consider Zanubrutinib.
  • 4yr
    There is no standard of care here. Consider Zanubrutinib monotherapy.
  • 4yr
    Given the diagnosis of amyloid, I would treat him with VCD.
  • 4yr
    This is a case of WM associated amyloid. I would test for CXCR4 mutations and given the MYD88 mutation would start BTKi, most likely zanubrutininb.
  • 4yr
    This is a case of WM associated amyloid. I would test for CXCR4 mutations and given the MYD88 mutation would start BTKi, most likely zanubrutininb.
  • 4yr
    Now that he has documented AL amyloidosis, I would recommend a light chain amyloidosis regimen such as Dara/Velcade/Cytoxan/Dex and refer him for stem cell transplant.

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