Lymphoplasmacytic Lymphoma that is not WM?

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Hello all, especially @jonyb, hope your wife is hanging in there. I got diagnosed about 10 years ago with both IgM (WM) and IgG kappa LPL. (and elevated kappa FLC) No symptoms to speak of and never needed treatment for them. Two years ago a third mutation, also IgG kappa, and more kappa FLC, showed up diagnosed in the end as CLL. Still no symptoms but started zanu treatment 4 months ago due to ever increasing new IgG/FLC and it's working well. I've been tracking the original LPLs at the IWMF forum https://groups.io/g/iwmfconnect/and suggest that you do likewise as the best source of relevant Ig LPL. So, here is what I have gleaned and my opinions. The rarity of non-IgM is a problem since only 75 cases per year are diagnosed in the USA compared to 1500 per year of WM.

First, solo Rituximab is not commonly prescribed to treat these conditions. Treatment options deserve their own posting, except to say that since both Ig types are similar B cell lymphomas, WM treatments are the first choice. And only treat due to symptoms that might include anemia. What symptoms led to your treatment?Bone marrow per cent is not the best way to keep track. Percentages don't track well with blood tests or symptoms.

The best way to track the LPL is testing total IgG and FLC, but supplement IgG number with SPEP and immunofixation which will give an M spike number representing the level of monoclonal IgG which is the culprit. For years my total IgG fluctuated between 1400 and 1900, upper normal range around 1500. But M spike ran around 1100-1300 indicating that most of it was monoclonal IgG kappa consistent with FLC kappa. IgM numbers ran 600-1200. BTW, in general kappa anything seems to be better than lambda.

Every WM expert I consulted formally or at educational forums were candid about the rarity problems of IgG LPL and scarcity of data but in the end all had success treating it like WM with anemia due to cancer cells in the marrow out competing normal cells for space. None had significant experience with elevated IgG causing significant problems. Jonyb's wife has unique, complicated issues compared to my uneventful journey. Never got into researching FLC much since they were only slightly elevated during my LPL days but really took off when the CLL IgG kappa clone showed up.

Enough for now.

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.

Hello all, especially @jonyb, hope your wife is hanging in there. I got diagnosed about 10 years ago with both IgM (WM) and IgG kappa LPL. (and elevated kappa FLC) No symptoms to speak of and never needed treatment for them. Two years ago a third mutation, also IgG kappa, and more kappa FLC, showed up diagnosed in the end as CLL. Still no symptoms but started zanu treatment 4 months ago due to ever increasing new IgG/FLC and it's working well. I've been tracking the original LPLs at the IWMF forum https://groups.io/g/iwmfconnect/and suggest that you do likewise as the best source of relevant Ig LPL. So, here is what I have gleaned and my opinions. The rarity of non-IgM is a problem since only 75 cases per year are diagnosed in the USA compared to 1500 per year of WM.

First, solo Rituximab is not commonly prescribed to treat these conditions. Treatment options deserve their own posting, except to say that since both Ig types are similar B cell lymphomas, WM treatments are the first choice. And only treat due to symptoms that might include anemia. What symptoms led to your treatment?Bone marrow per cent is not the best way to keep track. Percentages don't track well with blood tests or symptoms.

The best way to track the LPL is testing total IgG and FLC, but supplement IgG number with SPEP and immunofixation which will give an M spike number representing the level of monoclonal IgG which is the culprit. For years my total IgG fluctuated between 1400 and 1900, upper normal range around 1500. But M spike ran around 1100-1300 indicating that most of it was monoclonal IgG kappa consistent with FLC kappa. IgM numbers ran 600-1200. BTW, in general kappa anything seems to be better than lambda.

Every WM expert I consulted formally or at educational forums were candid about the rarity problems of IgG LPL and scarcity of data but in the end all had success treating it like WM with anemia due to cancer cells in the marrow out competing normal cells for space. None had significant experience with elevated IgG causing significant problems. Jonyb's wife has unique, complicated issues compared to my uneventful journey. Never got into researching FLC much since they were only slightly elevated during my LPL days but really took off when the CLL IgG kappa clone showed up.

Enough for now.

I am only 2 weeks into an expected 4 to 6 month course of treatment with Rituximab- Bendamustine. No change in my peripheral neuropathy as of now. Hopefully it will not get any worse and we hope for some improvement. The PN is the only symptom I have. Full neuro work up could find no other cause so we are assuming the high light chains are causing the axonal dysfunction seen on NCS and EMG. The goal is to get light chains down from 400 to a normal level. (About 20 I think)

@jerry48 Just had my kappa free light chains measured one month after starting the rituximab/bendamustine combo. The level went from 400 to 100 which was very encouraging.
I also was able to plantar flex my left foot enough to raise my heel up to about a normal level while standing on one leg. Right leg hasn't joined the party yet.
The 2 hematologists I have seen thought that any improvement in my PN could take 6 to 18 months once FLCs were lowered to normal. But this seemed to be partially guesswork because they hadn't dealt with, or rarely dealt with, a presentation like mine. Just hoping the progress continues.

@jerry48
I love your good news! Hoping it keeps on going!
I’m curious what your kappa/lambda flc ratio was before treatment?

Mine is very low at only .1

@jerry48 Hi Jerry thanks for your reply. It is a relief to finally find others with similar symptoms and condition, however I am sorry you all have this condition. Anna has a very steady IgG level in UK figures 16 to 19 g/l, however her kappa free light chains have been steadily rising from around 50mg/l now to around 90mg/l, kappa lambda ratio now over 25 if I remember. Her symptoms suddenly got a lot when the FLC rose above 65. We have suggested that hers is probably light chain driven as this seems to be the active element but we struggle to get them to even check them. NCS/EMG is normal for Anna. We both really hope treatment works for you, let us all know how it goes. Thanks again for your reply.

@jonyb
My kappa Light Chains were @ 400 initially. One month after my initial treatment with Rituximab and Bendamustine the level dropped to 100. Now, one month after my second infusions LC are down to 45. I have a return of almost normal plantar muscle strength in my left foot, getting a little better in the right. Numbness persists but sensitivity to cold has improved.
Meds tolerated well. Obviously I am feeling encouraged. No longer having any leg pain.
To summarize my diagnosis is LPL that is not MD, Other than some mild anemia, Kappa light chain elevation the only abnormality in blood work.

@jonyb
My kappa Light Chains were @ 400 initially. One month after my initial treatment with Rituximab and Bendamustine the level dropped to 100. Now, one month after my second infusions LC are down to 45. I have a return of almost normal plantar muscle strength in my left foot, getting a little better in the right. Numbness persists but sensitivity to cold has improved.
Meds tolerated well. Obviously I am feeling encouraged. No longer having any leg pain.
To summarize my diagnosis is LPL that is not MD, Other than some mild anemia, Kappa light chain elevation the only abnormality in blood work.