I was diagnosed with SMZL in May 2010 at age 46. I underwent CVP-R chemo for 4 months and I have two years of Rituximab maintenance that ends in June 2012. The C is cyclophoshamide, the same that your mother is taking.

My diagnosis seems similar to your mother's although there is a lot of detail about b-cell malignancies that can be different between patients. I also had a freakishly large spleen and bone marrow involvement. My spleen was the about the size of a ream of copy paper. It took up half my abdomen and had pushed all my organs to the right side. So you see, it looks the same.

I've learned a lot about SMZL and NHL in the past 16 months. There is a lot to learn about and I'm sure you're full of questions. Let me see if I can guess at some of the questions.

SMZL is a malignancy of the white blood cells, namely B-cells. One of the malignacies is that they don't die. The spleen collects them to dispose of them but since they don't die the spleen continues to grow collecting more and more. This makes it unlike a solid tumor form of cancer where it destroys organs and spread to other organs. It also tends to be an indolent disease, meaning slow growing.  This is both good and bad. Slow-growing means it generally isn't urgent to treat, but because conventional chemo kills cells by interrupting their cell division, it can be hard to keep the drugs in the body long enough to catch all cells during division. Slow-growing means slow-division. So this means that SMZL is generally not considered curable. Incurable shouldn't be looked at as a death sentence though. If all goes well your mother will respond well to treatment and that will give her about 7 years of remission after which a new treatment will be selected and the cycle will continue, probably until something else take's her life. As ironic as it sounds, thinking about other threats to life is hopeful.

Treatment can go well, if it does the spleen can shrink rapidly. Mine shrunk in just three weeks. I was prescribed gout medicine so that the massive number of dying b-cells didn't hurt my kidneys. I'm glad for this since a few litres of cells died in those weeks. After the treatment her spleen should be back to normal and in good condition.

If treatment doesn't go well, they may stop the treatment early and switch to another stronger treatment. I think splenectomy is reserved for when chemo can't be used or doesn't work.

Life expectency is a complicated thing and not many studies have been done with SMZL. You have to understand the statistics and what median means. Google for Stephen Jay Gould's "The Median isn't the Message". It does a great job of giving hope with regards to statistics.

Another factor about statistics is that the published studies often are based on old patients. To obtain the median survival rate they have to wait until half the patients die. This can take over a decade. Considering the study may have been published years ago, it's possible the patients were diagnosed 20 years ago. A lot has happened in 20 years. SMZL was misdiagnosed then or diagnosed late and treatments weren't as good. So you mother has 20 years of advancements in medicine to improve her lifespan.

The other thing to think about with SMZL is that half the patients used in the studies were 65 or older. At that age, especially 20 years ago, people could have likely died of other issues, and the studies usually don't care what they died of. So a patient stunned by his diagnosis could be hit by a bus on the way out of the doctor's office and his/her death would skew the results of the study.

My view of survival is loosely based on fact and conjecture, but I believe that a person who is otherwise healthy, not at risk of death from other illnesses and that responds well to treatment should probably expect 20+ years of life. As a younger, healthy (but overwieght) person I figure that heart or weight related issues are probably more of a threat to me than SMZL.

It's also not clear how people die of SMZL. Some (15% or so) transform into an aggressive form and while many will be successfully treated others may succumb. I figure at some point SMZL stops responding to all chemo regimens and the patient's immune system becomes compromised. I can only thing that some non-related infection or disease takes advantage of a weakened immune system. Like most people who have AIDS die of some infection I speculate that is the case for SMZL patients. That is unless heart disease or a speeding bus doesn't do it first. This is heavy speculation so please consider it as such.

Chemo has a wide range of side effects. There are some good treatment options for dealing with them. I use http://nhlcyberfamily.org as a very helpful resource. Although it doesn't have much on SMZL, the forum participants have lots of experience dealing with side effects.

Go ahead though and ask anything you like.

Hi I got diagnosed in Nov 2009 I was 39. I thought my weight loss was due to healthy eating,walking and hard work- turns out it was Gerald. His full title Gerald the B*#¿$¥d as I named him,was my giant spleen now residing in Addenbrooks. He was 7lb 3 when removed the size of a baby the pictures are great! I thought work was making me so tired but apparently Gerald was working overtime trying to clean my system. My surgeon once all the tests had been done and I had had a couple of unscheduled hospital stays put me to the head of his surgery list. Gerald was liberated. I have had a slow recovery back. Am not working because I really struggle with energy levels but I remain positive and determined to live a long life with my wonderful guy who I hadn't really been with long and has supported me. I am not scheduled to have chemo yet as my body is in self remission so long may that continue! Due to my lack of spleen I am susceptible to infection and am currently in week four of an annoying,draining cold. Any cancer diagnosis is crap but a slow growing,rare one is quite lucky. Hopefully with advances in research they may stumble upon a cure for those of us in this elite club. Until then I am and I hope your mother will continue to laugh lots,eat great food,drink fab wine and vist lots of places. Life is for living I am not gonna let this mild inconvenience get in the way. I just need my energy and the economy to improve so I can get a little job :-). Take care

Hi Flynn31

Thanks for your uplifting words. It's an honour to have a member of such an elite club among us! Welcome to Cancer Chat.

Jane

Ok so it's been awhile. I hope everyone is doing ok..I've had lots of highs, eaten great food,drunk a lot of fab wine and seen some amazing places.. Still in remission so that a great thing. However I obviously thought life was too easy because I thought I would get a second disease- haemolytic anaemia...this is the one causing all the problems. Making me even more tired due to the antibodies munching on my immature blood cells..my lack of immune system definitely keeps me on my toes. Going through a slump right now,not quite as positive,virtually housebound,government have taken my benefit and insist I go to work..would love to but 80% of my day I feel like crap. Muddling on regardless and hoping I can find myself again I seem to disappear a little more every day. Just want my life back. Until then I will eat great food and drink fab wine and see as many amazing places as I can xx

Hi All, and especially Mrs Ems!

I hope things are going well! I was diagnosed a month ago with SMZL at the age of 30. Its weird having a condition which usually effects older people. In some ways though its helpful as I can see what may happen as I get older.

I am trying to raise awareness of it with my blog http://spleniclymphoma.com/2013/07/10/the-purpose-of-this-blog/

I wish everyone the very best on here and if you are interested in what I am going through, or want to add some comments to my blog, or even help me write a post or two, please contact me.

Otherwise I will keep checking this post!

Marcus

Hello all,

I was just this week diagnosed with Splenic Marginal Zone Lymphoma.  I have the added complication of Acquired Angioedema which was the presenting symptom that led to the cancer diagnosis.  This makes me a very rare statistic.  Only ~200 people in the USA have been diagnosed with this particular complication; definitely part of this elite club.  HA!

I'll begin treatment with Rituxan soon.  I'm curious about the treatment.  I can't find a lot of info about it.  How successful is it?  Any info on life expectancy?  Side effects?  Any input is appreciated.  Thanks!

Hi just read your post, hope your mum is ok , and treatment is working for her , love too hear how she is doing . I was diagnosed 3yrs ago with smzl , I have had a splenectomy in June this year , it weighed 7lb, I feel a lot better without it,  I have not been offered any chemotherapy, I am back on watch and wait again , take care of your mum , Chris.

Hi,

I was diagnosed with a generic small B cell Lymphoma in October 1999 aged 49. It was Stage 4A, advanced but no symptoms.

Treated initially with Chlorambucil, five months later needed further treatment with CHOP. CHOP got me to July 2004 when it a Splenectomy seemed a good option. Spleen was 1.2Kg when it was removed, biopsy resulted in a more specific diagnosis of Splenic Marginal Lymphoma. It was twelve year later before I needed further treatment. Other than lifetime antibiotics, splenectomy has been a very good option for me.

Last treatment was July 2016, this time Rituximab. No side effects, everything going well so far, there has been no resultant lifestyle impact.

There is no usefull data for to relate  to but the option to retreat with Rituximab is still there. I am almost 18 years into this, still working full time, run 8Km several times per week and put this particular cancer in my nuisance bucket..

Hello Mikey, I'm Judith and have this week been diagnosed with SMZL with Acquired Angioedema also. I had to persuade my Haematologist that there is a connection because at first he was totally disinterested, but I insisted that he investigate and indeed he has admitted there is a link. I have had 8 severe bouts of Angioedema over the last 2 years and various doctor I've seen have not picked up on the connection, it was only this week when I was diagnosed that I started googling to look for further information, and found that here is indeed a link albeit a rare one. What I would like to ask you is whether your angioedema attacks have stopped now that you have had treatment, because my doctor could not give me a definite assurance, just a 'possibly'. I am having a splenectomy in 3 months time. Thanks.

Hello Judith,

I'm happy you finally have a diagnosis.  YES!  There is a definitie connection and it's extremenly rare.  There are fewer than 200 cases in the literature worldwide....I still can't wrap my head around those stats.  Anyway....I am happy to say that post treatment I have had no angioedema attacks.  Before diagnosis I experienced approximately 20 severe abdominal attacks in a year's time as well as many random body swells, including a facial swell (which is the episode that finally got me a diagnosis).  

Are you going to have Chemo or Rituxan therapy?  You mentioned that you will have a Splenectomy.  I'm curious why your Dr. wants you to have the surgery?  Rituxan therapy resolved the SMZL for me and I have been in 100% remission since October 2014.

Having said that, you should be aware that my results may not be your experience.  Even though it sounds like we have identical diagnoses, everyone is different.  You may not respond to treatment in exactly the same way I did.  There are many unknowns with this illness and the rarity makes it difficult to study and draw any conclusions.

If your doctor is still dubious, suggest reading work from the following researchers:  Dr. Sandra Christiansen, Dr. Bruce Zuraw and Dr. Marc Reidl.  These are the Doctors at the USHAE Center at UCSD, San Diego in the United States.  All three doctors are considered world experts in the field of Hereditary and Acquired Angioedema.  If fact my case study was published by Dr. C. last year.  I'll find the citation you'd like and you can share it with your doctor.

Good luck to you!  Please feel free to ask questions and please let me know how you progress!

Michelle