WM / MZL

Posted by jswv @jswv, Sep 10 7:38am

Hi,

I’m 43 years old, male, and was just diagnosed via bone biopsy for nhl, with the differential lpl/mzl.

From what i understand this is a grey zone diagnosis. I’ve had myd88 testing but that if I am correct isn’t always positive, and is then called myd88 wildtpe. I have had consistently elevated igm 423 and suppress iga 57 and igg 700 since march 6 when igm was 382 iga was 63 and igg 900.

My symptoms started a year ago with tinnitus a peripheral neuropathy, 60 pound weight loss in 2.5 months, night sweats. By January February new symptoms started, and have been increasing ever since: facial flushing, vertigo, trouble forming sentences during intense episodes of all the symtoms flairing, confusion, time perception issues, and uniliateral right side edema from foot to face. Vision problems. Foot neuropathy, turning purple than numb.

I’ve had two biopises one in a clavicle lesion and one para aortic node, both came back with identical clones and gene rearrangement. Spleen is normal on pet, no other biopsies performed.

I have not shown an mspike however my pet progression, showed a rapid increase in suv of both sites over 3 months.

The first hematologist diagnosed me with EMZL, which i know is impossible as there is no enlarged spleen and no tissue has been taken other than the node and the bone marrow. Having bone marrow infiltration and one left para aortic node biopsy would only allow a Nmzl diagnosis.

My symptomotolgy is and has been consistent with WM. At a 2nd opinion I was told an mspike can appear at any moment, but I was also told mzl does not move as quickly as it seems to be by pet scan progression and worsening symptoms, and rising quantitative igm with increased igg/iga suppression.

The 2nd opinion doctor is just giving the diagnosis of “MZL” without subtype. He has told me mzl is always indolent but wants to start therapy now with rixataub monotherapy.

At the first and only visit last week he gave me a “pulse” of oral dexamathasone for 4 days. After day 1 my symptoms all went into the most intense flair I’ve ever had and stayed flaired even after. Chest pains, intense edema, trouble breathing, flushing to the point of being purple, at points my neck swelled so much I had to hold my head back to breath.

With matching cd profile, it’s my understanding that these differentials are a gray zone. I understand myd88 being negative isn’t suggestive of wm, but it’s not exclusionary. The mspike which I’ve been told can appear, suddenly, seems to be the only way to differentiate. However, my symptomotolgy which began a year before a diagnosis of nhl, combined with my age, young for either type, and the rate of progression, doesn’t seem to fit neatly into MZL. I noted the reaction to the steroids to my doctor, and haven’t heard anything from him.

It seems logical to me at this point to keep testing for a few weeks, (my igm last went from 382 to 423 in 5 days). His plan is to treat as “MZL” for 8 weeks and see if it works. Which he informed me it might not.

I’d love any advice here, because it is difficult for me to listen and read his notes that contradict themselves, “urgent treatment” “8 weeks, not aggressive” “highly symptomatic” “too symptomatic for a lpd” .

Getting sick before the diagnosis for a year I know is not good. I don’t feel like I have time too waste, while I get sicker, and I’m getting no rational definitively for either diagnosis. My symptoms which all well documented since 2024 don’t seem to be considered at all.

Has anyone been in this situation before? Am I wrong to think perhaps starting a smzl specific first line treatment, and then waiting a total of 4.5 months since my last pet scan, and “seeing how I do” has a higher risk profile than testing for an mspike once a week for a month.

I have very unusual presentations of both differentials, and at and age that is rare. No matter which, the watch and wait period was missed, but in that time i have a severe case of cellulitis (2016) almost lost my leg from scratching a mosquito bite, 2013 I had an inginal lymph node mistaken for a hernia and had surgery I didn’t need, then a fever of unknown original 3 days after the laprospic surgery.

This Friday I am meant to go in have iv dexamathosone and rixitaub, I had a severe reaction the the first, and even after flagging to the doctor and np nothing seems to have changed.

It seems like the risk is all on getting mzl wrong, with no risk on getting wm wrong for the next few weeks.

Would love some advice, as I went a year with symptoms not considered carefully instead just seen as “symptoms”. In march when an immunollogist did the first ig panel due to my specific symptom constellation and it came back as it was, and I was tested for every autoimmune disease that could cause elevated igm and igg/iga suppression negatively, waldenstroms was left as the potential cause, due to my unique symptoms. 7 months later a bone biopsy revealed that very diagnosis differential. It is hard for me to understand the doctors rational in just going with mzl, no sub type and a treatment used most commonly for splenic mzl which i do not have.

Thank you,
J

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I am so sorry to hear that you are dealing with all of this. You did not say where you get your treatment. It sounds like you have already obtained a second opinion. You may need another one. I believe it is important to have confidence in your diagnosis and treatment plan. My 65 year old husband has been diagnosed with Lymphomplasmacytic lymphoma (LPL), but not Waldenstrom's. His biopsies also show marginal cell or LPL. He does not have a high IgM, but has high kappa light chains and mixed type hemolytic anemia. No other symptoms. We live in the greater Seattle area and receive care at Fred Hutch and MD Anderson. Our consultation with a lymphoma specialist at MDA sent us to a myeloma specialist. It turns out that my husband's CD markers align more with myeloma than lymphoma. His treatment is with meds for myeloma. He has seen improvement in his labs and his hemolytic anemia in the first 3 months of treatment. However, we understand your frustration. We are on a wait and see basis and don't easily fit into any category. If I knew after our first biopsy, what I know now, I would have pursued care at Mayo Clinic or Dana Farber because they see the most cases of these rare situations.

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I am so sorry to hear that you are dealing with all of this. You did not say where you get your treatment. It sounds like you have already obtained a second opinion. You may need another one. I believe it is important to have confidence in your diagnosis and treatment plan. My 65 year old husband has been diagnosed with Lymphomplasmacytic lymphoma (LPL), but not Waldenstrom's. His biopsies also show marginal cell or LPL. He does not have a high IgM, but has high kappa light chains and mixed type hemolytic anemia. No other symptoms. We live in the greater Seattle area and receive care at Fred Hutch and MD Anderson. Our consultation with a lymphoma specialist at MDA sent us to a myeloma specialist. It turns out that my husband's CD markers align more with myeloma than lymphoma. His treatment is with meds for myeloma. He has seen improvement in his labs and his hemolytic anemia in the first 3 months of treatment. However, we understand your frustration. We are on a wait and see basis and don't easily fit into any category. If I knew after our first biopsy, what I know now, I would have pursued care at Mayo Clinic or Dana Farber because they see the most cases of these rare situations.

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Thank you for your comment, and I’m sorry to hear about your husband. I am in NYC, so traveling to farber or md Anderson or mayo would be difficult for me.

In ny I have been treated so far at two hospitals with excellent reputations, but seem baffled by my differential. Combined with my age, my specific differential is a hematological grey zone, and unfortunately, it’s making getting care impossible. There is zero wieght to mzl in my case besides the cd markers, which are identical to lpl/wm. Symptomotolgy is textbook WM, and the appearance of them more than a year ago is what kept me going to doctors to try and figure out what is wrong. The fact that I got the IG panel, was purely due to me. I sought out an allegorist immunologist to see if I was allergic to something suddenly. My symptoms which I learened after Waldenstroms was brought up in march but was told it would be very unlikely, and they didn’t think I had cancer at all, and elevated igm are hallmarks of it. This was in march. The fact that in August I was not only told I have nhl, but the differential was wm/mzl seems like an impossible coincidence. Even so, it is not like I want either, all I want is to truly exhaust testing to the point where, and it could happen, they have to guess. With both cancers how you treat it matters. How you begin to treat it REALLY matters. It is beyond my comprehension that I have documented symptoms going back more than a year that that flair together, and have gotten worse together, and are indicative of one of the differentials, and not at all the other, that it is not considered. I’ve learned a few things so far about people like you husband and me, and what we are facing. 1) (maybe this will give you some comfort) my symptoms from around November on have basically left me disabled. I am out of breath making the bed, unilateral while right side edema, non stop tinnitus, confusion, in a flair the inability to speak. If your husbands labs are off, but he still feels relatively ok, I wouldn’t worry so much because with cancers like ours symptoms really matter. Having them is usually the trigger to start treatment and get out of watch and wait, I’m not sure about myeloma, as I actually have a clavicle bone lesion from my cancer, but was told I don’t have myeloma.

2) if he’s on meds and improving that is good. That’s the goal. You got through the bottle neck I’m stuck in, and your husband should definitely feel good about that! It’s certainly still scary, but i can tell you from experience. I got a pretreatment for treatment and it made me sicker. It ironically is for myeloma, but I was prescribed as steroid pulse at a very high dose. It has but me into potential kidney failure. I found this out Friday from the hospital. The Dr who prescribed them, didn’t show up to what was meant to be my first infusion. He also didn’t respond to 10 or so calls emails etc, when I started having a severe reaction on day 2 of the 4 days or the entire 6 days after. So really take a treatment and improvement as a very good thing.

3) This is the most important. The medical system is broken. It doesn’t work correctly. Throw in a rare disease and the battle is uphill. The best thing you can do for yourself is download and read every single after visit summary, google what you don’t understand, and then use your own common sense to see if it adds up. You don’t need to be a doctor to do it either. I’m a younger generation, and more internet savvy but I can assure you, it’s all not that complicated. Doctors for the most part will tell you don’t google blah blah. However, what google does is just give you acceess to research information etc, that before the internet was unavailable to the patient. You and your doctor can read the same guidelines, the most up to date discoveries etc. The difference is you are 100% more likely to actually do that. Dr.s have to many patients and in the end for most, it’s a job. It’s not a calling. So do not be afraid to research everything about myeloma. You can even just copy and paste those cd markers for your husband’s pathology, and check for yourself what they match. WHEN INFORMATION BECOMES MORE FREE, EXPERTISE MEANS LESS.

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Hello @jswv

Some links I have squirreled away:
- Have you joined the Waldenstrom's Foundation -- https://iwmf.com/
---They have a list of docs you can reach out to directly (https://iwmf.com/wp-content/uploads/2023/10/IWMF_PHYSICIANS_DIRECTORY.pdf)
- Another is the CLL community in https://healthunlocked.com/.
- https://www.dana-farber.org/cancer-care/treatment/hematologic-oncology/programs/waldenstroms-macroglobulinemia
- Waldenstrom's Macroglobulinemia Clinical Trials Group [http://www.wmctg.org/]

I can't provide any other helpful advice as I haven't experienced your symptoms. I do hope that you find folks that can help you to get better!

Regards.

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