Primary cutaneous marginal zone B-cell lymphoma (PCMZL)

Hi Medgabi,
I’m sorry to hear about your husband’s diagnosis.
If you don’t mind me asking what part of the body had the 5 spots? Are there they itchy and are they non painful? Were they nodules? Any symptoms before or after diagnosis? Any fatigue?
Is that the only treatment offered?
I couldn’t find the FB page.
Thanks

Hi!

Thank you. He has 2 on his forearm and one on his elbow on the same arm. And then one on the back of each shoulder. The largest one is about 1/2 of an inch and the others are all much smaller and flat. And the largest one has regressed significantly the past 6 weeks without treatment. They are not itchy or painful. They were not nodules. He has had no symptoms. No fatigue. The largest one flared up in March which is why we went to the dermatologist and then discovered what it was. They have given us 3 different options - intralesional steroid injections, IV rituximab or watch and wait.

Hi!!
1. What did the patches of skin look like? Scaly white skin or pink or red skin? What colour are the flat patches? Did your husband think they were a kind of psoriasis skin type complaint then…… except none were itchy? and not painful? No symptoms? What was he thinking the patches of discoloured skin were when he made an appointment to see a Consultant Dermatologist? Has he any thoughts on causation?
2. Was it the dermatologist who gave 3 options? Or, a Consultant Haematologist?
3. Has he had a CT or a PET scan? Will they be taking bloods regularly?
4. Which option are you thinking of going with? I don’t understand option 1 of option 2? Please explain if you can
I had 1 isolated red nodule on my forehead. I had it excised. No other treatment. Bloods every 6 months. Watch and wait only.
Many thanks

Take Care

They’ve all been more pink/red. Most of them look like skin blemishes/small pimples? So we were never alarmed by them. The larger one on his forearm did look more like psoriasis or like a fungal patch. His primary pointed it out at his yearly physical and prescribed a steroid cream and a antifungal cream which ultimately made it angry and it flared up and grew more puffy and red and bumpy which alarmed us so we made an appt with a dermatologist. They’ve never bothered him though. And we’re not sure what the cause is.

We’re working closely with both an oncologist and a dermatologist that work together treating cutaneous lymphoma in their clinic at UCI. They both have provided us with those options.

He did have a PET/CT last month - all was clear. He had blood work done as well - all was good, I’m sure they will take blood again in the future, but haven’t discussed a timeline.

Intralesional steroid injections are steroids that they inject directly into the skin lesions to shrink them.

IV rituximab is a immunotherapy drug that they administer intravenously at the infusion center. It would be 4 weekly infusions for a month. This medication specifically targets a protein on the abnormal b cells and kills them. However, the downside of this medication is that it also kills the “normal” B cells so he would be immunocompromised for 6 months to a year after the last dose. Doctors are offering this as an option because he has multiple spots on his skin.

If it was just one spot like yours, excision or laser radiation to the spot would have been an option.

I’m hesitant to do the immunotherapy, just because of the side effects and am leaning more towards the steroid injections and then watching and waiting.

We meet with oncologist again in 3 weeks after she presents his case to her co-workers to hear their recommendations as well.

I wish I could upload pictures here to show you.

Hi!!
I thought my pinkish/red raised nodule on my forehead which started as a dot and grew to 1cm X 1 cm was a completely innocent pimple. I thought to myself I won’t squeeze this. I’ll leave it and it will just disappear naturally. When it didn’t and continued to very slowly grow over 3 years ……. I made the appointment with a Consultant Dermatologist and was told it was a Basal Cell Carcinoma. It was excised. 3 monthly later I was called back and told the biopsy came back and it wasn’t a BCC. I was stunned/extremely upset. I was told I was a 1 in 3 million person and this PCMZL is extremely rare. Cause unknown. So it’s good to chat.

I was over the moon to learn your husband’s PET/CT scans were completely clear. So happy.

At my last bloods 2 weeks ago I was told by the nurse that the bloods tell them nothing?! I’m going to ask my Consultant Haematologist what exactly that means? I will let you know what she says.
I wish you could upload pictures too. I don’t know what a fungal patch would look like? Is it completely white? And does it have a completely flat white scaly grainy texture? Does it look like a flat white scaly grainy perifungal wart? Or is it a flat pinkish/red scaly grainy texture? Thank you

I pray all is really good news from the oncologist in 3 weeks. Please let me know.

I personally wouldn’t have the drug. I personally would investigate myself the injections more fully before making a decision on them. These are extremely difficult personal decisions to make.
Take Care. Kindest thoughts and regards

Oh my gosh! That’s wild! They did look like small pimples. Nothing about its presentation ever alarmed us.

From my understanding, the blood work won’t show much unless there is systemic involvement. Liver/kidney markers, red blood cell, white blood cell or lymphocyte changes. Or elevation of LDH (lactate dehydrogenase) which measures the amount of cell turnover in the body. But with just skin involvement, there are likely no changes. Which is good!

Yes usually fungal patches are white and scaly and itchy and flat. His were never like that but we really didn’t know what else it could be.

I will keep you posted on what we decide!

Yes the injections are the more conservative route, but I also want him to have the best chance of complete remission without relapse if possible.

I wish it was just a single lesion like yours, then treatment would likely be more straightforward.

Hi!!
Yes wild! How is it possible that 1 isolated very faint dot on your forehead would slowly grow over 3 yrs & be diagnosed as PCMZL?! What? How? Why? So many unanswered questions……

Thanks for your reply. You are very knowledgeable about PCMZL. The small reddish/pink pimples that your husband had & no symptoms……. I’m speechless…. it’s so random ……. so rare…… no one knows a lot about it imo.

Keep me posted/updated on what you decide & likewise.

This may sound like a silly question….. but ……Is your husband really worried about it? How serious is PCMZL in a 1 - 10 scale do you think? Not sure what to think?

I can’t find the FBpage

Take Care
Kindest regards

I will keep you posted!

And not a silly question at all. When he was first diagnosed, we were very shocked and worried and didn’t have much information on it. My husband was definitely anxious (as was I) and it was a very hard few weeks as we were navigating through testing and doctor appts.

Now that we know it is just on his skin and there is no systemic involvement we are relieved. And have been reassured by his doctors.

We’ve been told -

1. Out of all of the lymphomas (there’s over 150 I guess?) that this is the “best” one to have

2. It is very lazy and slow growing and very treatable

3. People do not die from this

And after all of the research I’ve done, I would probably rate it around a 3-4/10?

It’s definitely something we’ll need to stay on top of and manage, but plan to continue living our lives to the fullest we can!

We have been trying to conceive for a year now. I’ve had two early losses. So that is our ultimate goal and are feeling grateful that we have the chance to keep trying!

I found the page but haven’t been accepted yet.

I just typed in “PCMZLers” and scrolled down until I found it!