IVHG V3-21; IGHJ FAMILY J4

[Sunfishjoy]

The vote is not in yet about my diagnosis--CLL or more likely SMZL. Meantime one lab reported this morning that I am unmutated and the gobble degoop in my title. I found one smzl article online that said mutation status is not prognostic for smzl (especially since rituximab) but would appreciate hearing from anyone who might help me understand what this means if it means anything at all. Thank you!

[Lizard28]

Hi, this must be awful waiting to be told results and then they come back with this confusion. I don’t have SMZL but have NMZL, I’ve had treatment but only had six weeks remission before it came back. Waiting for my results second time round I was told it was just suspicious and she ordered further testing to be done and then it came back positive again. Can I ask if you been having Retuximab for another illness? This is what they give most people after chemo for maintenance treatment. Unfortunately they said I could not have it for my rare lymphoma but reading on here a lot of people do get it. I’m under a rheumatologist as well and I know retuximab is used to treat RD. I guess it has to be very clear what the diagnosis is before they can get the correct treatment for you but wish they wudnt tell you a half diagnosis, so frustrating. I’m on watch and wait this time so I’m hoping it doesn’t develop into B symptoms and get a few years before treatment is needed. Sorry I didn’t really answer your question but hope your doctor finds out your results quickly from your biopsy. All the best

[Sunfishjoy in reply to Lizard28]

Hi. No, I haven’t had any treatment at all. As far as I knew I was in perfect health until April this year when all this testing began. Glad you are W and W and hope you stay there for a very long time.

[Andilynn]

It is very hard to wait for results. I was diagnosed with SMZL in the fall of 2014 and began treatment with Rituxan immediately. Two years of maintenance with Rituxan followed and I remain in remission. I meet with my oncologist and have blood work done every six months. Feel free to contact me with any questions.

Best,

Andi

[Sunfishjoy in reply to Andilynn]

So glad you remain in remission. Was your spleen enlarged? Did it go back to normal size? Mine is 16 cms.

[Andilynn in reply to Sunfishjoy]

Yes, my spleen was 22 cm, the size of a full term baby. I also had enlarged nodes in the chest, abdomen and pelvis. I had large bruises all over and broken blood vessels in my eyes all the time. I didn’t feel sick though and until I had routine blood work, didn’t realize how sick I was.

Rituxan worked quickly and within about six weeks, my spleen was normal size.

[Sunfishjoy in reply to Andilynn]

Wow. Fast and great response. I have no nodes enlarged. Just enlarged spleen and lymphocytes in bone marrow.

[Andilynn in reply to Sunfishjoy]

My bone marrow was involved as well, hence the stage IV. That’s very typical with SMZL. I have lingering side effects of Rituxan, but it was the drug I needed and it worked.

[SuzeJc in reply to Andilynn]

I thought I had CLL with stable bloods, but we just found out my spleen is 25 cm and it's likely I have SMZL. I feel completely in the dark all over again. Where abouts are you based? have you been told about other treatment options other than Rituxan? What if Rituxan doesn't work or cannot be tolerated? I presume there are no clinical trials? Has your specialist told you anything about prognosis? Do people tend to do well on treatment? I'm in my 30s with two children under 3 who need me around.

[Andilynn in reply to SuzeJc]

I was given 4 rounds of Rituxan and two years maintenance on it (finished in Nov. 2016). The FDA has just approved a biosimilar version of Rituxan. I suspect your doctor will talk with you about it. I am unaware of any clinical trials for SMZL. It is a rare cancer, but responds well to treatment most of the time. Some patients are given a combination of Rituxan and chemo therapy. I opted to go with just Rituxan first to see how it would go and that worked for me. I live in Ohio and was treated by doctors at the Cleveland Clinic. Due to insurance, I have other providers keeping tabs on me now. Feel free to contact me any time. This is not a journey anyone wants to face, but try to stay positive. Eat well and exercise if possible. You are not alone...this group is here to care and share.

Best,

Andi

[SuzeJc in reply to Andilynn]

Thanks very much for getting back to me. I'm glad the treatment went well for you and I hope that you get a long remission. Please stay in touch.

[SuzeJc in reply to Sunfishjoy]

I thought I had CLL for 2.5 years and was told it's progressing really slowly and I'm years away from treatment... suddenly, 4 weeks ago, we found out my spleen is 25 cm and I need treatment and it looks more like SMZL. Have you been given any further information? Where are you based?

[Sunfishjoy in reply to SuzeJc]

Hi Suzejc. I'm in nyc and undergoing diagnosis now. One world expert believes I have smzl, not cll, because I am cd5 negative and cd19 &20 bright. My spleen is (was?) enlarged to 16 cm. I'm seeing a second world expert this afternoon. I'm afraid I can't be of much use to you right now because I am just learning about mzl. But I am very glad to have the chance to share info. with you.

[SuzeJc in reply to Sunfishjoy]

How did your meeting go with your second opinion?

[Sunfishjoy in reply to SuzeJc]

Hi and thanks for asking. He said he also thinks I have Splenic MZL and should be W&W yet be checked every three months for now. He wants to do a CT in October but I will check with main dr first as I’ve had so many scans. Meantime I feel good and will do my best to enjoy the rest of summer. I hope you do too.

[Sunfishjoy in reply to SuzeJc]

PS I did join the Facebook smzl and it’s a very active and informative group.

[SuzeJc in reply to Sunfishjoy]

I'm glad you're feeling good and focusing on enjoying the summer. My consultant is keeping me on watch and wait for now but I have 2/3 bone marrow involvement and also a very large spleen so he said I won't be on watch and wait for long. What's the exact name of the Facebook SMZL page? A number of people have recommended it but I cannot find it. I can find one group with 3 people in it only.

[Sunfishjoy in reply to SuzeJc]

The exact name is “SMZL Support Group”

[SuzeJc in reply to Sunfishjoy]

I've found them now but only by someone sending me a link. Another group kept appearing on my searches that only has a couple of members! Thank you!

[curlscurls]

I didn't know IVHG mutation status came with variations of gobble degoop. I don't think mutation status is used to determine treatment, at least not in smzl. No one cared or asked me what my status was on that. When I asked if it was in any of my results and had missed it, they still didn't express interest.

Do they use alphabet soup at their doctor conference lunches, to feel right at home?

[Knitter79]

What I SMZL ?

[Sunfishjoy in reply to Knitter79]

SMZL, Splenic Marginal Zone Lymphoma, is a little cousin of CLL who shares many traits but differs in severity and location of the cancer cells. Most SMZL patients are treated by CLL specialists. Many are first diagnosed with CLL but blood tests show the presence of CD20 which is absent in most people with CLL. I am currently on Zanubrutinib and doing well with a normal spleen size and no other symptoms.

[Knitter79 in reply to Sunfishjoy]

Thank you for the description