My mother was diagnosed with lymphoma of the spleen. Can lymphoma be isolated in one place? How risky is Rituxan in a 75 year old with diabetes, hypertension and a 60 year history of smoking?

Yes it can, in its early stages, although my understanding is that this typically isn't the case for splenic marginal zone lymphoma (SMZL), which is what I'm guessing is your mom's diagnosis.

Rituxan seems to be the drug therapy of choice, probably because SMZL is a B-cell lymphoma that expresses the CD20 antigen on the surface of its cells-- precisely what rituxan was designed to target.

As for risk with Rituxan, there's nothing ostensibly in the prescribing information that details risk factors for what you're mentioning, although there are warnings concerning the heart, lungs, liver, kidneys and other major organs.

Sorry I couldn't be of more help.
Ross

Stacy-
In lymphoma, bone marrow involvement automatically means stage IV disease, and often a splenectomy is the preferred way to go in patients who can handle it. There's unfortunately a bit of controversy over the best way to treat SMZL, as this paper indicates.

Ross

I was diagnosed in May 2010 with SMZL and the prescribed treatment was chemotherapy with Rituximab. That shrunk my spleen significantly in just 3 weeks (doc said back to normal, but I have doubts. I think it returned to normal after 15 weeks.) The reading I've done says that a splenectomy *was* the treatment, but it seems now that watch and wait or mild chemo is quite common.

Thank you very much for your response! I am still confused though. My husband is 39 years old and not a surgical risk, so is the article saying that he should have the spleen removed or should he do some other treatmenet first? The article seems to say in the beginning and middle that he would be a good candidate for spleen removal, and that would have a good success rate, but the last sentence seems to contridict that. Any clarificiation on the article would be nice. THANK YOU!!!

I am 44 (45 today) and, other than being overweight and NHL I'm in good health. My spleen was 11 inches high, 9 inches wide and over 3 inches thick. It grew down below my belly button and to the right past my belly button. On the CT scan it had squeezed my intestines and stomach to the right of my body. Almost the entire left was spleen. I'd say that counts as larger than a cantelope.

The spleen is part of the immune system. I read it is useful, not like an appendix. Although I'm not sure but it appears that after a couple of years other organs take over for the removed spleen.

In my case my bone marrow was involved, this is very common in many NHLs. I think it means that the bad cells are caused by the bone marrow not the spleen. So removing the spleen only treats the symptom not the cause. I was told that the spleen filters bad blood cells. I'm also told that SMZL is caused by mutated cells that don't die. I was also told the spleen grows to hold the filtered cells and that may cause other cells to be filtered as well, that was platelets in my case. My blood counts showed low platelets (70) and high LDH before treatment. They returned to normal after the first chemo.

The chemo I had was mild in that I could work and didn't lose much hair or have other really bad effects. There were effects, but no nausea, most effects were caused by prednisone, a steroid, which made me jittery and made it hard to sleep. The chemo was CVP-R. CHOP-R is another chemo commonly used in NHL. Its the same as CVP-R except with one additional drug. That drug is toxic to the heart and causes more hair loss so it is not usually used as a first option.

As I see it splenectomy is not the best solution. Having cvp-r worked well for me. My spleen shrunk considerably in just three weeks and completely in 15 weeks. I do admit that I think my spleen is back growing again (3 months after treatment). I can feel dull pinching in that area. I'll know more next week after the onc appt.

Another thing to consider is that you shouldn't feel rushed to decide. Just make sure your husband has "indolent", that means slow growing. If so you can seek another opinion.

I was told the biggest risk factor of a large spleen was it rupturing. If your husband is punched there, is in an accident or is otherwise hit in the spleen it will more easily rupture.

I scuba dived with that big spleen for likely a long time so normal activity probably won't be a problem.

Also keep in mind a lot has changed in the last few years. Check the date on that article. Rituximab has extended remission and chemo dosages is much better understood.

Don't be afraid of CVP-R chemo. It wasn't at all like what I thoguht. I was somewhat tired and had mild allergic reactions to the rituximab, visually it wasn't apparent I was having chemo. The only days I took of was the days os the infusions. I was infused on Fridays so I could rest the weekend. I work in an office so the work isn't physical, that may have helped.

CVP-R is probably more expensive than a splenectomy. I'm told the cost of rituximab is $3000 per dose and there are usually 14 to 16 over 2.5 years. Add the other drugs and administration it could be $5000 every round. I'm in Canada so the total cost for treatment was $200 in prescription drugs. Does cost factor into your doctor's or your decisions? That may be why a splenectomy is preferred.

It's good that you and/or your husband are taking an active role in diagnosis and treatment. In my experience doctors only seem to work on a patient a few minutes every so often and can often lose sight of the patient's unique big picture. You are the one that has to tie things together, bring long-term history. Simply asking appropriate questions like "why splenectomy over chemo" may prompt a doctor to re-think.

As for bone marrow transplants, as I understand it they aren't done much any more. Stem cell transplants are the big thing and the cells can come from the patient (Auto SCT)(if they can be filtered from the blood stream) or from another person (Allogenic SCT). They are tough on the patient because it involves high potency chemo to kill off the entire immune system. This puts the patient in a precarious position where an infection is quite deadly. In an Allo SCT the patient gets donor stem cells and the procedure is safer because the patient's immune system doesn't have to be completely killed. The donor cells kill the rest because the donor cells see the entire patient's body as an invader. This also means that there can be chronic rejection issues.

In your case I don't think you have to worry about SCT.

You can also consider joining http://www.nhlcyberfamily.org/ it's a very active list and the site has tons of good information. Reading and learning is your best weapon against this. And make sure to maintain that continuity of information that doctors often don't have.

Thanks. that is our #1 question....Why is he recommending to remove the spleen over trying chemo first?

I do feel frustrated because I feel like I am gathering info from others and the internet and I feel like our doctor should be the one tellingus all this.

Did you make it to your appt yesterday. I hear the weather has not been good there. Ironically, we were in the mid - high 80's yesterday.

Most of the information I got was from sources other than the oncologist/haematologist. NHLCyberFamily.org has been very good in understanding NHL. I've also read a lot on-line. It's taken a lot of reading and thinking to get where I am. My onc isn't a good source of information and the 15 minutes we have every few weeks isn't long enough to get a lot of information.

I recommend getting all the test results including bone marrow biopsy results, blood results and scans. Blood results can be tracked from before treatment to see where they go. I also got the CAT scan. This is part of the continuity that we as patients can contribute to the process.

The blood results were particularly important to me. I was told that during chemo I should avoid germs since my immune system would be depressed, that included scuba diving, something I do as often as I can. I avoided diving for months until I got my test results and found that my important blood counts prior to treatment, when I was diving all the time, were not far off what they were before each chemo cycle. So I started to dive again the weekend before each chemo. The caution from onc now seems overly cautious. I never caught a cold during chemo, nor got an infection from diving. That included a 12 hour flight to Abu Dhabi on business (and squeezing in as much diving there as possible) for 14 days just after a final chemo. I was scraped by the reef, stung by jelly fish and cut superficially on a steel shipwreck during that visit with no ill effects. I also ate in local restaurants (risking food-bourne issues.)

I assume your husband is otherwise healthy and could handle chemo. (the effects were like a week-long mild/moderate hangover, not the kind to swear off drinking forever but the kind where you need aspirin.) Poor health would be one reason to avoid chemo.

Another reason would be if his "version" of SMZL (there seem to be many factors) doesn't respond well to chemo. My b-cells have CD-20 markers which makes them easy to target by chemo. Having the CD20 marker is pretty common.

As you can see from my experience life can go on pretty normally with SMZL. I hope I hear good news from my onc this afternoon so I don't have to worry.

This good news for me may be good news for you.

I just had my three month post-chemo checkup and it went well. The doctor palpated for my spleen and declared it still normal. The sensation I'm feeling, she says, is something else.

I took the opportunity to ask more questions. She says that SMZL is rare enough that there aren't any statistics. The stats she has givin me are for follicular which she says (and has said) is a similar indolent NHL. One notable exception is that SMZL has a very low rate of transformation to an aggressive form. In fNHL transformation is a 50-50 chance.

She used a former patient as an anecdote. The patient moved away (thus "former") but she received an email recently. The patient was diagnosed with SMZL at age 88 and was put on Rituximab only, 4 doses. She is now 96 and still doing well. It was minimal treatment with great success. She thinks that is typical with SMZL and suggested that a 10 year remission can be expected.

I also clarified the basic mechanism of SMZL. B-cells are created in the bone marrow, mature somewhat there and migrate to the lymph nodes (of which the spleen is one) where they will mature into a plasma B-cell when they are exposed to antigens. During the process the cells divide and differentiate into different types of B cells somewhere in this process the resulting cell's DNA is damaged and the cell's natural death clock is disabled. The spleen isolates these cells and when it grows it also traps other healthy cells like other B-cells and platelets and red blood cells.

The malignant cells don't die naturally, but they also don't replicate often. This is why this type of cancer is slow growing. It's also why it can't be cured. Conventional chemotherapy works by killing cells while they divide. Since these cells don't divide often it is very difficult to catch all of them dividing while the chemo drugs in a patient's system. This is why Rituximab is useful. As a biological agent (and anti-body) it is not a drug but living cells that stay in a patient's system for months killing B-cells that have the CD20 marker. With many types of NHL the malignant cells have this marker.

I'm guessing radiation can't be used because the cells are actually dispersed throughout the body and irradiating the whole body would be deadly. Radiation works with solid tumours because they are localized and radiation can be focussed to attack only malignant cells.

I hope that this gives you more hope with your husband's prognosis.

I am glad that your appt went well. Happy to hear that your spleen is still "normal" size.

That info is helpful. At this point, any information that help us to understand this is. You seem to have a great doctor who explains things very thuroughly and understandibly. You are lucky. We are going for a 2nd opinion on Jan 4th and then maybe to another doctor at the Los Angeles Kaiser, which is the biggest one around here.

We just want to make sure that we are doing the right thing. We are really re-thinking removing the spleen! It seems as though he would still have to do chemo after anyway. It seems as though the chemo and the rituximab are the way to go!

As for your treatments, how often did/do you go? Like is it once a month, three days a month, etc. And for how long are you/did you go for those tr5etments? three somths, six months, a year?

THANK YOU VERY MUCH!!!!

It seems the details of this are so complicated that it's hard to explain. My onc really doesn't get into the detail I want. This visit I must have shown enough knowledge that she opened up. It's also the last time I'll see her for two years if all goes well. I'll be seeing a nurse practitioner before each rituximab maintenance.

I was treated with CVP-R which is cyclophosphamide, vincristine and prednisone with rituximab. After the chemo there is rituximab infusions every 3 months for two years.

The original plan was for 8 treatments three weeks apart. It was expected that within 6 weeks my spleen would have shrunk considerably. It happened in three weeks. After 5 treatments they reduced it from 8 to 6. In total I was "in chemo" for about 4 months. The week after each chemo was like a hangover. Sinus headache, tired and jittery. The two weeks following I was fine. The worst part was that it was the entire summer "in chemo" and it just happened that every long weekend except one fell right after a chemo treatment.

My hair thinned (most said they couldn't tell) but came back when treatment ended, my taste went funny after each treatment but returned quickly. Towards the end my finger tips when numb (peripheral neuropathy) and it took three months to return. Despite losing what must have been 10 lbs of spleen I ended up gaining 25 pounds during chemo. No physical nausea at all, except the combination of jitters and fatigue gave a mild sense of nausea. Half way through I ask for a sleep aid prescription and she prescribed lorazepam. If he ends up taking prednisone I recommend lorazepam, it will allow him to sleep well at night and reduce the fatigue.

It was mild enough that only the people I told could tell I was in chemo, although I kept the couch company the weekend after each treatment.

The rituxumab is the biological and tends to provoke an allergic response. I had only a minor reaction, my sinuses would feel a little stuffy for a week. Others get a big reaction when it is infused and it needs to be done slowly.

I still don't know if your husband's SMZL is exactly like mine, but if my onc thinks it will be 10 years between chemo, I'd take that over spleen removal.

Stacy if you want to email privately look me up on Facebook. I'm the only John Van Ostrand there.

Thanks for all the info!!!!

I'm the one with John Candy's picture.

Also you might be interested to check out my SMZL blog. It might give you some insight to SMZL and chemo. http://www.vanostrand.com/smzl.