Just to start off I want to say I have had bad news about my health. I have Splenic Marginal Zone Lymphoma, a non Hodgkin’s Lymphoma. This is a cancer of the lymphs and is non curable but has a generally good prognosis in term of long term survival. I have posted a number of blog posts in chronological order about the journey from a routine blood test to knowing what I have and what it has taken to get there. I will continue to use this blog as a vehicle for my thoughts and analysis of it and what impact it has. There are currently 7 posts, so feel free to read back through them and find out more.
I have taken the decision to blog about this not to get attention for myself but for several other reasons. The main one is that there are millions of other people out there who have or will develop health conditions and if I can only help one of them who is going through something similar to myself then this has been absolutely worthwhile. Some Conditions have a lot of information about them available, others are relatively rare with only a handful of papers. Mine falls into the latter. I have only just discovered I have it and having looked online there is little or nothing about it. I wanted to change that. I also wanted to show how the diagnosis process feels to someone with no medical knowledge as you go through it step by step.
The other reasons to write this are that 1) I like to write, I have written a few unpublished books and enjoy being creative. 2) This will be a method I have for coping with what I have. I would imagine I will write about all sorts of things in my posts, but initially I will concentrate on where I am at with diagnosis and how you find out you are ill.
If this causes any of my friends or family distress, I can only apologise. Please remember the reason I am doing this and help me with my journey. Fingers crossed it will be a long blog with not much happening in it. That in itself would be a real win.
spleniclymphoma 
Fair play to you Marcus. With a positive attitude it well help you achieve all sorts!
My best wishes and good luck are with you.
I’m tipping my hat to you so hard it’s causing tsunamis in the Pacific. All the best from us in the Arctic, you rock!
Hi Marcus,just found your blog and enjoying it.My name is Neil and like you was diagnosed with SMZL in 2009 and also like you Iam a lot younger than the average patient with this disease,I was 44 at diagnosis.I went to have a blood test for cholesterol as I thought at my age I should be checking things like this out,got a call from the Doctor the same as yourself and was told my WBC was at 50.after this I was misdiagnosed with a disease called CLL from the local haematologist.Went for a second opinion at the main hospital with a very well regarded Professor in Blood cancers and had more tests and ended up with the SMZL diagnosis.I am also in the UK living near Chester.I have been on watch and wait for the 4 years having 6 month checkups.My WBC count is now at 100,but I feel fine and don’t suffer any abdominal pain due to the spleen ,which has stayed at the same size since diagnosis.Talking to my doctors I think it will be retuximab treatment when it is needed which has a very high rate of getting us into remission and not being a chemotherapy you avoid a lot of the side effects.
I wish you well and will keep in touch
Regards Neil
Hi Marcus,
I was diagnosed with SMZL in Nov 2007 when I was 57. So far I have had no treatment just regular blood tests every 2 -6 months depending how my counts are doing. It took me a few years to believe what I was told at the initial diagnosis – I would die of something else not the SMZL. I hope that you are keeping well and that this post will help you.
By the way my son has been asked to donate some stem cells to the Anthony Nolan trust. Does anyone know if my SMZL will prevent him doing this – especially as SMZL is not supposed to kill us?
Regards Suzy
Hi Suzy, Good news. 7 years later and still looking good is a great sign! Fingers crossed I will be able to say the same.
To the best of my knowledge there is no reason your son won’t be able to donate, its not hereditary (as far as any research had proven) so unless he has it already it should be fine. After all its a random mutation of a cell, so shouldn’t be a problem.
Hope that helps and thank you for the message!
Best regards,
Marcus
Hi Marcus,
I was diagnosed with SMZL in 2010 with a spleen the size of a ream of paper. I responded well to chemo (CVP-R) and was in remission after the first cycle. It’s been 4 years now.
I found Stephen J Gould’s essay The Median is the Message a very good source of hope when I was worried about the survival statistics. I recommend reading it to get a positive view of statistics.
Hi,
My brother (42) was just diagnosed with SMZL (in bone marrow and neck lymphnodes (neck was insignificant). He had his spleen removed – because he had B symptoms (fever,sweats, weight loss). He’s recovering from surgery now and supposedly starting Rituxan early January – 1x/week for 4 weeks. We will know for sure in December when the re take the PET scan and Bone Marrow biopsy. Does anyone have a similar story?
We also feel we are at the best specialist possible in our area (NJ)
I finally found a blog by others diagnosed with SMZL. I am a 64 yr. old woman, otherwise healthy, diagnosed with SMZL 10-13-14. I have had hemolytic anemia all my life, but other than marginally out-of-normal-range blood counts, it wasn’t a problem. Spleen 18 cm. I had six Rituxan infusions and now, 60 days later, no sign of change. Dr. is recommending splenectomy. I got a second opinion and it’s the same recommendation. I am concerned, as I read from another posting, about infection from surgery. I will have to have traditional big-cut surgery as my doctor feels that my spleen has encroached on other organs and can’t be simply extracted. I am concerned about the length of recovery as well as whether or not there will be real benefits to the surgery. I am largely asymptomatic and my spleen can’t be felt through my skin and it isn’t making me full. I don’t find very much about SMZL and would be grateful for any insight that can be provided.
To have the spleen out or not? I am especially concerned because I have hemolytic anemia, will always have that, and my immune system is already compromised. Quite a quandry.
I was 44 when diagnosed and treated for SMZL. My spleen was 30cm but it shrank back to normal after one cycle of CVP-R chemo. Is there a reason they are opting for surgery instead of chemo?
A splenectomy has an impact on your immune system. I’m not it sure but I think that eventually the remaining lymphatic system compensates for the loss.
I also was told that the spleen is enlarged because it is trapping malignant cells and those cells aren’t dying because of the malignancy. Removing the spleen always seemed like it only solves a symptom and not the cause. I suppose it’s like a treatment with chemo in that the cells sequestered by the spleen are removed but again why not chemo?
Incidentally, for me chemo was like having a moderate hangover for a day or two every three weeks. No nausea and no noticeable hair loss. Prednisone, a steroid was the cause of the hangover since it interferes with sleep. My stubborn refusal of sleep aids meant several cycles of hangover. I should have used them earlier.
Other chemo regimens are more harsh but still not like the stereotype we are used to.
I hope you find this helpful.
Thanks for so quickly reading my post and responding. I had six infusions with Rituxan (over a six week period). Also got steroids and benedryl and an few other things. No shrinkage in my spleen (a dainty 16 cm) or significant improvement in my blood. It is now 9 weeks later and still nothing. I suspect I will get another spleen ultrasound to see if the bad boy has shrunk. My oncologist and another with whom I consulted both feel the splenectomy is the way to go (of course it’s not their spleens that are coming out). Both MDs concurred that Rituxan is the way to go as opposed to chemo or Rituxan+chemo. Studies I have read point to Rituxan as the first line of defense with usually stellar results. Far less than stellar for me. I would like to know if others with SMZL and hemolytic anemia have had the spleen removed. I am not keen on taking antibiotics the rest of my life (I am allergic to many of them already) nor do I wish to be under house arrest for fear of illness and infection due to the weak immune system. Any comments for you or any other SMZL ‘owners’? Thank you!
Hello,
At last…other folk with the same cancer..!!
I thought I’d write my post as I literally stumbled across this site during a frantic web search. I’m 43 and have just been told that I have Marginal Zone Splenic Lymphoma…devastated is not the word. My spleen is 23cm long and only the past few weeks been causing me an ache. The Haematologist has said that removing my spleen is not an option as the Lymphoctyes are in my Bone Marrow so not resolving the problem. I’m waiting for the results of my Cytogentics to determine which type of Chemo…
I only went to the Doctors because I had a rash on my leg which didn’t go away other than that I felt fine and still do… then the journey began that was Feb 15, I’ve not got an uncomfortable feeling/ache in my chest which could be because of my anaemia/enlarged spleen or something else (.
Would appreciate any response to see how you are doing.. anything really!
Helen
x
Have emailed. Sorry to hear this, it gets easier, let’s chat more. x
Hi John,
So good to chat to other people….means a lot…
Similar age then eh at diagnosis. Would you mind if I asked you some questions about your treatment and what to expect and how things have been since then?
Marcus I’m just gonna read your email now..
Thanks
Helen
Aside from the rash I had the same symptoms and was 45 at the time. My spleen was bigger about the dimensions of a team of copier paper.
I had CVP-R chemo and within one cycle my spleen was back to normal. I’ve been in remission now for over 5 years with no issues.
You’re young and SMZL has a good prognosis.
Good to find this blog! I was diagnosed with splenic lymphoma a year ago at age 52. I had an initial 6 rounds of Rituxan. I had an initial very bad reaction to the first treatment and required additional steriods for the two treatments following. Rituxan has helped bring my speen down from 24 cm to 14 cm. it took about 9 months for it to shrink to this size. My blood counts have rebounded except my white cell count hovers slightly above or below the minimum 4.3. I am hoping it’s due to the Rituxan still. I am on every other month Rituxan maintenance treatments for two years as my doctor said studies show this helps. I feel great except seem to be experiencing a less sharp of a memory. My blood sugar also has risen. I have been able to drop to 1/4 dose of steriods and think that will help. Hate living with this but heard the prognosis is good. I just was to see my white cell counts rise and my spleen size at least maintained. I would be happy to share more if anyone is interested. I am curios to hear from anyone who has finished two years of maintenance treatments and how things are going.
As you can see from my response to Helen that I also have SMZL. I was diagnosed at age 45 with a 34cm spleen. After one round of CVP-R chemo (CVP with Rituximab) it was back to normal (no size given). I underwent two years of rituximab maintenance without any ill effects. It’s been over 3 years since that ended and I’ve gone from 3 month checkups to 6 months. Everything normal since then. There was one time my LDH was up but that was likely because of a recent infection.
I was told that since my spleen and counts reacted quickly that a long remission was expected. So far, so good. I’d say based on that your R-only treatment bodes well. As I understand it every subsequent treatment will need to be stronger and since you started with the lightest treatment you have another iteration of treatment than I may have. As time goes by better treatments will be created and we will live longer. It sounds dark but I expect something else will claim my life.
I’ve heard of “chemo brain” in which people who have had actual chemo complain of memory and fuzziness. I’m naturally bad with memory so I probably wouldn’t notice any degradation. At our ages (I’m now 50) you can expect all sorts of degradation caused simply by our age.
Good luck with things. Ask for my contact info if you want to keep in touch.
Hi John, thanks for the reply. That is great you responded so well to treatment(s) and are doing well several years into your diagnosis. I like your optimism. I would really appreciate some added discussion and take you up on your offer to keep in touch via regular email.
Rich
Hi R, I see John has just replied. There are a few people here who can share experiences with you. I personally can’t, as I am in the watch and wait camp, but I am also sure I’ll be there at some point. Prognosis is very good, so I have read. I read a lot of articles in medical journals when first diagnosed and we are in an ok boat all things considered. Keep me updated on your progress!
Hi Everybody…..I have SMZL at least the doctors are 90% sure of that. Seven years earlier at 50 I was diagnosed with Chronic Lymphacetic Leukemia after a bout of shingles (a year ago and still have nerve pain) my spleen enlarged 25cm X 19cm I am opting for a splenectomy as opposed to chemo/rituxin. The reasons being I would like an exact diagnosis and only a spleen biopsy will give that, there was no guarantee that my spleen would shrink enough for me to get back to life, there is risks with surgery and having no spleen but I feel this is my best option at this time. I was just wondering if anyone out there has had a splenectomy and how things went for them, and how they are doing now. I would love to hear any feedback. thanks.
Chrystal
Hey everybody an update on me and my SMZL…. I was originally diagnosed with Chronic Lymphacetic Leukemia 7 years ago but a year and a half ago that all changed,I broke out in an severe cas of shingles (hospital for 23 days) my spleen enlarged quickly and furiously and a new diagnosis of SMZL was given, .on March 31st I had a splenectomy to remove my very big spleen who I had named “Bob my bump to the left” . My year and a half of turmoil had somewhat come to an end. It was not an easy decision and it took me a few months to finally decide. I was concerned with the chemo and rituxan route that at some point my spleen may enlarge again and hinder my life activities. The spleen had stopped me from working and enjoying the simple life that I was leading. It literally got in the way of many things. Anyway my surgery went well I spent 4 days in the hospital and have been recovering nicely ever since. I was able to do some things that I hadn’t done in awhile…..bending and putting on my own socks and tying my own shoes was something I always took for granted but with “Bob” as big as he was I couldn’t do that…..but once again I can. My first oncologist appointment post splenectomy was enlightening and brought my spirits to a height I had not been to in awhile. My oncologists words were inspiring as she said to me” I am so proud of you, you made a difficult decision and have come out of the surgery with flying colors. I believe that the splenectomy was the best decisions for you and you did the right thing.” My blood levels are improving and things are leveling out slowly but I will get there. The best news of all is that I will return for a routine checkup of blood work etc. in the fall (October) and if everything is as good as it is now and I still am continuing to feel well then I can begin the transition back to my job which I love and miss so very much. The return may not happen since I work in a classroom and the infection rate is very high. We will have to wait and see how I can handle infections and sickness that are so common in these places. I am very optimistic that if I do my best to stay healthy and think positive my return to full time in my classroom will happen. (fingers crossed for me please). This journey has not been an easy one for me in fact at times I was scared, frightened I would perhaps die, I became depressed because physically I could not do any activities and I was bound to my house for periods of time to avoid flu season and the shingles outbreak happening in my community, I struggled with why this happened to me, and I felt alone, and nobody understood what was going on in my life. Then I discovered blogs, websites, and online support groups that made a tremendous impact and change in my life. To realize I was not alone in this journey in my crazy life I was ecstatic it brought me comfort and calmness to know I was not facing this bump in my road of life alone. To hear other peoples comments on how they handle their SMZL was encouraging,it helped me realize that I can do this. In all of this I realize that my diagnosis of SMZL is not what defines me or my life, it is not in charge of me, but in fact, I am the adult in charge of my own life. As far as my diagnosis of SMZL I have learned to smile and wave and appreciate all of the good that is in my life. Yes my SMZL is on my mind and in my thoughts, but, the good news is, it is not all I think of anymore. I have learned and am still learning to adjust to my new normal and so far it is working. I no longer sweat the small stuff, I am grateful for everyday, I appreciate my family and friends and all the support and love they bring to my life.I am no longer reluctant to ask for help and I am willing and happy to receive it when I get assistance when needed. I have found and reconnected with my spiritual side and enjoy the comfort that it brings to me. I now meditate and breathe and take the time to connect with my soul and my inner self.I make and take time for me and I love it. I look forward to the blogs websites and support groups I have found they bring me up when I am down they give me the strength and they comfort my fears. It is somewhat ironic to me to know that people having the same diagnosis/disease as myself is where I have found most of my relief and comfort I reached out and people just like myself were there to help. I know that it is not always going to be sunshine and rainbows, there will be struggles and setbacks but I can honestly say that after a year and a half I think I’m doing okay. I wish everyone who is living with SMZL the best of the best, may there be sunny days and smiles and love and support to help you with your diagnosis/disease. I pray everyone has a health team they can depend on and whatever decisions you make for yourself for treatment it will be what works for you. Not everyone will choose a splenectomy but for me it was what worked. I know there is no end to my SMZL but at this moment I am the one who is in charge, I am living my new normal, I will take one day at a time, and I will put on my big girl panties, throw on my cape, adjust my tiara, and fight this SMZL head on I won’t quit, I will continue to win this fight. Good day to everyone and keep fighting because…….you’ve got this…..we’ve all got this. We can all do this. cheers to all, Chrystal
Hi
Having recently been diagnosed with splenic lymphoma , I am at the ‘what the hell’ stage. I am 51 and although initially diagnosed with Hairy Cell Leukaemia after further investigation the consultants have given this latest news. I am currently watch and wait with only a WBC of 15.4. It really is difficult to find information about this condition and although I am more tired than I used to be I feel ok. I am very stressed about it but I think it takes a long time to get your head around it. My consultant has told me that a splenectomy will probably be the way to go further down the line, however, having read other posts I think this may be a last resort!!!! Will keep you posted. Good luck to everyone x
Hi Sue,
I know exactly how you feel. I was told hairy first and then splenic. I was 12ish WBC. Four years on I am still fine, now on a 26 or so. Didn’t need a spleenectomy either. The condition you have is better than most, which once you get to that stage you might be thankful for. For now you’ll just be really angry, confused and sad. You can only work through that.
All the best though and keep me posted.
Marcus
Hi Sue,
It’s been 7 years since I was in the “what the hell” stage. I did a lot of reading and a lot of thinking after diagnosis. Maybe I can fill you in with what I know, the Cliff Notes so to speak.
SMZL seems to take at least two forms from my uneducated knowledge and the small sample of people I know who have it. There are some whose spleen grows (splenomegaly) and blood counts drop then there are others who have astronomical counts. I’m the former.
The reason for both of these is that with SMZL the malignancy with white blood cells is that they don’t die. This is very unlike stereotypical cancers where malignant cells reproduce rapidly. So rest assured SMZL is not a typical cancer. I hope that brings you some relief. In some people the spleen traps these odd cells sequestering them until they die, which they don’t, so the spleen grows large to them all. At the same time that reduces your WBC counts. With the others the malignant cells stay in the blood stream and result in astronomical WBC counts.
There are a few treatment options. Removal of the spleen (splenectomy) is one. This used to be a first-line option but it fell out of favour about the time I was diagnosed. First line treatment now is either chemo or an infusion of antibodies. And of course lack of treatment is an option, that’s watch and wait.
The chemo options I’ve seen are CVP-R or R-CHOP. They are similar except that R-CHOP has one more chemical than CVP-R. CVP-R is preferred since it’s mild. Patients usually take a day off work every three weeks for this. Hair loss is barely noticeable and side effects are very manageable. This is not the stereotypical chemo. I gained weight and looked normal and felt normal most of the time. For 5 days of the 21 day cycle the Prednisone had me jittery, like too much coffee.
What I’ve seen lately is the use of rituxiumab, an antibody infused via intervenous. The only side effect is an allergic reaction that some people have. The benedryl they use to combat that makes me sleepy for a few hours. So a half-day of napping during the infusion and I’m normal for the rest of the three week cycle.
With the current treatments I figure the future consists of treatment, followed by years of remissions (7 now for me), then watch and wait, when it comes back treatment again and the cycle continues. Each subsequent treatment will be less successful and will result in using stronger treatments. Hopefully you’ll die of something else, like old age. Given that the median age for diagnosis is about 65, most actually die of age-related things. We are young enough that there’s a chance that today’s treatments will stop working and severe treatments like a stem cell transplant will be needed. Delaying that as long as possible is the long-term strategy so the longer you spend in watch and wait the further away that is. So W&W is a good thing.
I chose the words “today’s treatments” specifically. Better treatments are being developed. We’ve already moved beyond chemo and spelenctomy as the only options. The antibody treatment is very effective and better antibodies are being developed. Even genetically specific treatments are being tested. These two are very promising treatments. So by the time you need a second treatment it might be something very new and more effective.
I figure maybe four more treatments is what today’s science has for us. That could be 20 to 30 years of remission during which time new treatments will be developed that push that back another couple decades. So keep up your health, you may be around at age 90 and later.
Keep up the good work! How are you feeling? I just went through 4 rounds of Rituxin after a stage lV SMZL diagnosis a month ago… what a shock
Let fight to find a cure and get more information on how to live well for a very long time.
Marina
I’m with you on that! So far I’ve been lucky and not needed treatment. Smzl is always stage four, but treatment that quick is a surprise, what were your counts? Big fan of the cure thoughts!