Hi, new to this board, I am posting for my mum, she will be 80 in April this year.

Twenty years ago she was diagnosed with a very rare (8 : 1 million) condition called Amyloidosis, we are in Scotland and she was lucky she received excellent diagnostics and treatment plans at the Royal Free in London which is the centre of excellence for Amyloidosis in Europe, our local hospital gave her the recommended treatment of high dose chemotherapy.  Thankfully she has been in remission for 15+ years but her spleen and kidneys were irreversibly damaged.  She was put on daily antibiotics for her spleen and kidney function was closely monitored.

3.5 years ago while at the Royal Free for a 2 day Amyloidosis check up with my sister she was sent home with instructions to attend her local hospital immediately on landing.  That night my dad was home alone and died of a heart attack.  Mums spleen was the size of a ream of paper, she was severely anemic and needed two blood transfusions and we had to go in the next day and tell her her husband of 55 years had died.  A few weeks later she had her diagnosis of SMZL.

She was treated with Rituximab, was hospitalised after the first dose for 10 days as her potassium levels were dangerously high, doctors don’t know why as it’s not a side effect of Rituximab.  After the potassium issues were finally resolved she continued the Rituximab treatment and tolerated it well.  Her spleen shrunk and everything has been ok for 3 years.

18 months ago her kidneys failed and she is now on haemodialysis 3 times a week.

Just before Christmas we noticed she had been losing weight and a routine blood test showed low platlets.  A CT scan between Christmas and new year showed a recurrence of her SMZL, she starts Rituximab again on Friday .

i feel more worried this time, she is nearly 80 and frail, due to past chemo she can’t have it again, a splenectomy operation is not an option for her.  She is on dialysis this time and the consultants don’t seem to know how/if this will affect her Rituximab treatment which successfully sent her into remission last time.  Has anyone here had multiple Rituximab treatments and remissions?  I guess SMZL is too rare to find someone with experience of dialysis and Rituximab too?

I am new to this chat as well, but thought I would share my experience even though I live in the U.S.  I was diagnosed at age 53 with SMZL in October 2012 at which point I had a massive spleen ("Frankenspleen")and 70% bone marrow involvement.  I had four, once a week, infusions of Rituxan and my spleen decreased in size immediately, much to my great relief.  I had side effects from the Rituxan, but only during the infusions and my fusion clinic managed them well.  My numbers didn't improve much though and my spleen began to increase in size again so I had five more rounds of Rituxan in April-May of 2013 and a splenectomy in May 2013.  Goodbye Frankenspleen.  I have been fit as a fiddle ever since.  Getting rid of that nasty spleen, which I was initially loathe to do, was the best decision.   My counts have all been good and I now only see the oncologist once a year.  I thought I had a certain expiration date when I was diagnosed and I worried constantly about dying before my sons were grown, but I don't even think about that anymore and they are now 18 and 20.   I still hold my breath when I go for my yearly check, but I know that Rituxan will be there again should I need treatment again.  Happy to answer any questions anyone may have.

Cheers to good health!

Amy

Thank you very much for this info.

I am a 37 year old male and was diagnosed with SMZL last July.

My symptoms at the time were persistent mulcers and upon investigation I was found to have a high WBC count.

My spleen was enlarged at 17cm and I have bone marrow involvement. 

I for the past 12months I have  bèem on watch a wait with regular blood tests. My WBC count has steadily risen to 34 with all other counts stable.

I know I will need treatment in the future and feel fine at the moment.

I have found it realy hard to find any current research and anyone in a similar position as me to talk to.

I have read everybodies posts and was wondering how you were all getting on?

Regards

D

Hello Dspoc82,

I suppose you have read through all the previous thread about SMZL, I have written a few pieces about my experiences with the disease, and at the moment I am feeling very well indeed having had 6 weekly sessions of Rituximab in October 2017. I started feeling better immediately and haven't looked back. Each one of my check ups since then (monthly for a year, now down to four monthly) has shown that my blood is normal. My Haemotologist says my SMZL will definitely come back sometime but cannot say when, but when it does I will have more Rituximab which will hopefully give me another remission. I try to eat as healthily as I can and lead as healthy a lifestyle as possible, as I feel sure this helps. Any questions, please ask away. Good luck, and hope all goes well for you.

Hi Judith

Thank you for your message.

please keep me up to date on how you are getting on.

regards

Dan

Hi Dan,

Thanks for your message. My next check up is in August, will let you know how it goes.

Regards, 

Judith.