WM / MZL

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