Lymphoplasmacytic Lymphoma that is not WM?

I have IgG-secreting Lymphoplasmacytic Lymphoma which, although somewhat similar to Waldenstrom's, is even rarer than Waldenstrom's. However, the general treatment of both are the same.

I was scheduled for 6 cycles of Bendamustine & Rituximab chemotherapy; however, only actually completed 5 of the 6 cycles. My 'hematologist/oncologist' team cancelled my cycle 6 informing me that the chemo therapy was basically totally ineffective on me. During the chemotherapy, my IgG paraprotein had stagnated and actually plateaued at considerably less than what was expected. Rather than chemotherapy, I was transitioned instead to the BTK Inhibitor Zanubrutinib. As Zanubrutinib is capsules, they can be taken daily at home eliminating the need to go to a medical site to be injected. I've been on Zanubrutinib for 25 months now and am currently considered stable as my IgG paraprotein levels are not currently progressing; however, these same levels are not decreasing either.

I have IgG-secreting Lymphoplasmacytic Lymphoma which, although somewhat similar to Waldenstrom's, is even rarer than Waldenstrom's. However, the general treatment of both are the same.

I was scheduled for 6 cycles of Bendamustine & Rituximab chemotherapy; however, only actually completed 5 of the 6 cycles. My 'hematologist/oncologist' team cancelled my cycle 6 informing me that the chemo therapy was basically totally ineffective on me. During the chemotherapy, my IgG paraprotein had stagnated and actually plateaued at considerably less than what was expected. Rather than chemotherapy, I was transitioned instead to the BTK Inhibitor Zanubrutinib. As Zanubrutinib is capsules, they can be taken daily at home eliminating the need to go to a medical site to be injected. I've been on Zanubrutinib for 25 months now and am currently considered stable as my IgG paraprotein levels are not currently progressing; however, these same levels are not decreasing either.

Hi! My husband is also in that small subset of patients that does not have typical WM with high IgM levels. He has Cold Agglutinin Disease which is related to his IgM, but his IgM levels have been within normal limits. He has high Kappa Light Chains. We live in Seattle and have pursued additional opinions because he has not been given a clear diagnosis. This summer we were told that he has LPL that mimics Myeloma. Last week, we went to see Dr. Castillo at the Bing Center at Dana Farber in Boston because we were at a crossroads for treatment. He has no symptoms. We are waiting to speak again with Dr. Castillo after all of his test results including a bone marrow biopsy are complete. He is MYD88 negative, but now we see he is positive for CXCR4.

@murray3
Thank you for sharing. I’m glad that you are doing ok- that is good, and hopeful news!
Docs don’t quite know what to do with us yet…but at least there are options. I am glad that I went to Mayo in October and have those experts on my team.
I had 8 Rituximab infusions (allergic reaction to each one, ugh) in early 2025 which did reduce the cancer in my bone marrow from 75% to 40%. Not as good as we hoped.
I’m not quite sick enough to start chemo yet.
Zanubrutinib has been mentioned as the next step.
Have you had many side effects?
Wishing you a lovely day.

So, after months of tests and biopsies I finally got a dx of kappa light chain LPL. My only symptoms have been a distal neuropathy in my lower legs.
Just started Rituximab and Bendamustine. Tolerated well. 4 to 6 monthly cycles anticipated. Hopefully light chains will return to normal and neuropathy will clear though this is uncertain.

So, after months of tests and biopsies I finally got a dx of kappa light chain LPL. My only symptoms have been a distal neuropathy in my lower legs.
Just started Rituximab and Bendamustine. Tolerated well. 4 to 6 monthly cycles anticipated. Hopefully light chains will return to normal and neuropathy will clear though this is uncertain.

@jerry48
Hi and nice to e-meet you. It’s so good to hear that you have a diagnosis and treatment plan. Wishing you all the best with BR.
LPL is so weird! My Lambda light chain is off the charts, opposite your Kappa situation.
Are you being treated at Mayo?
I have a local oncologist in Kansas and go back/forth to Mayo-Rochester (6 hour drive), as necessary.

We are a small group, I hope we can be in touch to compare notes and provide support.