Hi Simon,

Welcome to Cancer Chat. I can see you have also posted in another discussion so I'm assuming you've had a search of the forum already - it seems like there aren't too many other similar discussions on here but hopefully you'll get a reply back from that one.

Either way, I hope that the forum can be of use to you, and we're always here for support if you need it, even if it's just to write things down.

Wishing you all the best,

Ben
Cancer Chat Moderator

Hey Simon, 

Yes, I have plasmablastic lymphoma. Started July last year. I have just relapsed. 

I hope you are still doing OK? 

I'd love to hear from you and  how you are coping physically and mentally.

All the best,

Sarah

Hi Sarah

Thanks for the message. Sorry to hear about your diagnosis and the relapse.

I'm doing okay although I have really struggled with infection so far in 2023 which was not the plan! 

I was first diagnosed in summer 2020. Physically, as I say infection is the main problem at the moment. I have a PET scan coming up imminently to see what position the PBL is in after the latest ongoing drug regime. Without infection, I have had periods of relative 'normal health' and have felt pretty good. Mentally I've been very up and down. That has tended to be in synchrony with the infections.
 

How are you finding it all? Can I ask what regime/regimes you've been on?  Feel free to ask me any more questions. 
 

Best wishes

Simon

Hi Simon!

Many thanks for your prompt response. I sometimes feel I have become a member of a very exclusive club that I joined involuntarily! There can't be very many of us around. I am getting my treatment in Nottingham which is the haematology centre for the East Midlands. It's a big clinic and I am their first case in two years....It feels like a dubious honour.

I am 57, very fit and healthy (worked in the fitness industry for 25 years before this) until I became rapidly ill with PBL in July last year. Large tumour on pancreas which also blocked my bile duct and caused jandice. I was given the EPOCH chemo regimen to which I responded very well (without too many vicious side effects) and was declared cancer free in Dec. I have been really well since then but at the beginning of April I got a lump in my boob which is the return of the PBL. I see the consultant on Wednesday to find out what happens next.

You sound like you are doing well if they can get a grip on those infections for you. I guess four lots of chemo is quite punishing on the immune system. Keeping fingers ctrossed you get an infection-free summer to enjoy and an excellent PET scan result.

I appreciate your invitation to ask questions :) Which chemo regimes have you been on? Where are you getting treatment? Where was your original tumour and where were the relapses? Between each relapse do you feel you've been back to your former health and fitness?

How do you manage with the uncertainty? In the last few months I have found it quite anxiety-making to constantly be vigilant about a cancer that could come back anywhere. It feels like every little thing - even a mouth ulcer - could be nothing .... or everything. 

For the most part I work hard to get the most out of every day, walk, exercise, be with friends and family, laugh, distract myself etc but sometimes the grief and terror can overwhelm me. I guess that's only human.

I am so glad to have found you! And likewise, please speak freely and ask as many questions as you want.

All the very best to you!

Sarah

Hi Sarah

No problem, I was keen to respond as soon as I saw the email pop up! I never knew whether a response would come! Yes, you have made it into the exclusive club! That's a very good description of it. Would be nice if we could leave a bit of a thread of info for others on here as there is very little as it stands..

I'm 36. Very fit and active prior to my diagnosis and was in pretty good working order! Even though I have been HIV positive since the age of 21. I wasn't allowed to start treatment in Berkshire straight away back in the early 2000's. So, basically, they waited for me to become sick, and I started to develop AIDS type illnesses before they started me antiretrovirals. Encephalitis being the main impetus. I have remained on treatment until present day. I feel this period without medication irreversibly damaged my immune system and even though it happened at a young age I never quite recovered. In addition to this my father died from AML when he was 45 (another blood cancer). Although PML and AML aren't currently known to be linked and AML is not deemed to be hereditary I feel that it is too much of a coincidence and that there is an immune weakness that has been passed down. In HIV lymphomas I think PBL counts for about 1-2 % so it is still incredibly rare. I’m also HHV8 positive. Like EBV it can play a part with the development of certain cancers and lymphomas. Mine does seem to be linked to the HHV8 and in the beginning I was treated directly for the HHV8 in the hope that this might help. I was treated with Valganciclovir for several months.

My lymphoma first presented in the small bowel. I had intermittent pain which felt like a sharp wind pain. But it was 100-fold worse than that and it wouldn’t clear like wind and would last for hours and hours. The episodes became more frequent accompanied by nausea, constipation and severe weight loss. Finally, a bowel obstruction in the ileum occurred followed by intussusception, I then had bowel resection surgery which removed the growth and some of the lymph nodes. After removal no obvious disease could be seen on the PET scan, but the spleen was slightly enlarged.

I started CHOP therapy at my local hospital a couple of months later, after they’d worked out what I had from the biopsy! But the chemo was more as a precaution. They didn’t think I'd tolerate the stronger EPOCH due to having an ongoing fistula infection. But even with the CHOP, the fistula contributed to neutropenic sepsis after each cycle of chemo. After 3 rounds they decided it wasn’t safe to continue. A subsequent PET showed no disease progression. A suspicious spot in my liver was ruled out to be a granuloma possibly related to the most recent infection. So, I was still in the clear Stage zero/1 PBL.

I had a mixture of good and poor health throughout 2021. I was able to work and had a physical job as a landscape gardener. However, the year ended up involving a lot of auto immune type symptoms and I was later diagnosed with muscular vasculitis. However, I’m now not sure whether this was just the lymphoma acting strangely. I remained stable to a point, from January 2021-January 2022. I started a treatment of dexamethasone to treat the expected vasculitis in Dec 2021. I don’t know whether you had steroids as part of your regime, but they really can be very effective. After completing a month of steroids. Things really started to go downhill for me and I got incredibly sick.

I then spent several months in a certian London hospital. I knew this was one of the best places in the U.K/ Europe for PBL with HIV (just to say that this was still NHS). While I was admitted there, they found that I had MAC lung disease which is like a non-contagious form of Tuberculous and I started treatment which I was told would last for 12-18months. Symptoms only improved so much on treatment and shortly after another PET scan and subsequent biopsy from my neck would indicate a relapse. This time the PBL was all over the place. Largely effecting the muscles which again really isn’t common so don’t compare yourself to me in this sense! At this stage my B symptoms were very much present. I was then to start 3 rounds of VTD-PACE.

In the spring of 2021, I had undergone surgery for what had become a very large fistula and had a colostomy fitted to allow healing after the surgery. This allowed me to have the stronger chemo. Saying that I had sepsis after every round again but just from other things! Another PET in July 2022 concluded CMR 3. (Complete metabolic response level 3) on the PET scan which I was pretty pleased about!

From July to the end of September I was pretty good, starting to enjoy life again and making plans and mentally preparing myself for an autologous transplant. Unfortunately, they failed to collect enough stem cells from me and by the end of October I’d relapsed again. Symptoms were mainly heavy flushing, sweating during the day mainly focused around the face and head (no night sweats) low energy, low grade fevers which became high grade over a few weeks and an LDH which was just jumping up rapidly each week. CRP also jumping up but not so dramatically. PET scan confirmed the relapse, and I had lesions all over the place again.

It was then I started on daily Thalidomide and Dexamethasone once a week. Symptoms improved quite rapidly I’d say. Within 48hrs the fevers had gone, and my energy levels began to improve. It was quite dramatic and astounding and I had so little in the way of side effects. This is similar to a regime for a Myeloma patient and the drugs are nothing new. Both diseases are similar, effecting the plasma cells or plasmablasts which become plasma cells, so it does make sense that they are treated in the same way, but this is/was a bit of an experiment.

I had a fantastic January. I joined the gym, started playing badminton, gardening, socialising more, doing my artwork again and upcycling, DIY and signed up to learning the violin! Unfortunately, by the time we got to February the infections caught up with me and they haven’t really let up. Is it the cumulative effect of the previous chemo, MAC, HIV, having previous cancer, taking steroids or having active cancer. Will find out more after this PET scan.

So, yes, the anxiety is not great is it! I’ve felt it at its worst, it can be hellish and crippling. It can make everything feel overwhelming. It can create some symptoms or accentuate others. But it is there for a reason and will get you to act on things and get help when its needed. It’s just a case of keeping it in check. Having the possibility of death really staring at you for the first time and the pain you may or may not experience along the way is scary. I have found it terrifying. Something has changed for me though. I have had some psychotherapy through Macmillan and also acupuncture. I’ve also had reflexology and massage through Sue Ryder. I also did a lot of mindfulness at the beginning of this escapade. It takes a lot of practice, but I found it could completely take me away for half an hour and could hit the reset button for a while when I would otherwise be climbing the walls. I also relied on lorazepam for a while. Don’t be afraid to ask the doctor for that or similar, that’s what those drugs are there for. I took it a lot for a while. But it’s a rarity now.

The following may not be everybody’s approach and some people may be actively against it. But if you can except the possibility of death and the fact yes, this disease may kill you. Then I have found this takes a lot of the worry away. We are all going to die, some people old some people young. In the U. K less so, but in other countries young people die all the time. Try and change your expectations of life. And don’t wait for things to get better start trying to enjoy it today. Try and make the most of the bad days and you are less likely to feel you are wasting time. This is not a full proof structure for me. But I feel being less worried about death helps you enjoy today, if you spend every day worrying about the aches and pains and essentially death you are wasting all those good days. Don’t worry, if something really doesn’t feel right, you will know and you will ask the doctor and if they don’t take it seriously. You will make sure they do!! And remember in dying you are not alone it’s the one very thing we are not alone in, we will all be in the same position.

Hope this is helpful. Might have gone over things that you already know, or you think aren’t correct so feel free to correct me or question what I’ve written! I hope I haven’t overloaded you with too much info! If you are to go on treatment, I’d be interested to hear what options the consultant suggests. Happy for you to share my case with him if you think helpful. Although I know the consultants share data across U.K hospitals anyway.

Best wishes

Simon

Hey Simon, 

I SO appreciate your writing such a detailed reply. Thank you so much for taking the trouble to do that. I had to look up for a few words and abbreviations! A language you never wanted to learn....

I am so sorry to hear you have had so many difficulties and hospital stays but - wow - how resilient the human body can be! I really hope you get some plain sailing from now on.

I saw the consultant yesterday but still no biopsy result so no course of treatment finalised as yet. I'll get in touch next week when the treatment plan is finalised. They have me on a 40mg dose of dexamethasone to try to arrest the development of tumours in the interim.

As for the anxiety and grief....

When I realised that had relapsed I just felt very sad .... at the very likelihood of not living out my possible lifespan with the people I love. You are right we all die, but when you've been in remission and hope has crept in - it takes some mental gymnastics and a bit of time to return to place of acceptance. 

The terror for me is not death but the manner of my death process. This lymphoma which apparently can appear anywhere, offers such a smorgasbord of unpalatabe potentials.

I too have had some counselling through Macmillan which was great. 

In terms of strategies to calm myself down, I am blessed to have worked as a rehabilitation movement specialist for the last 25 years. My breath practice and movement practice have saved me through all this. The breath practice to calm me through undesirable medical procedures in particular! I move every day, outside in nature whenever it's not raining, And I speak my gratitudes and my hopes/manifestations. You are right. It resets. I always feel way more able to govern my thoughts and emotions afterwards.

I was able to stay strong and fit during chemo last time which really helped I think... and meant I was really able to make the most of my remission time.

I really so appreciate this interaction. So good to be in touch with someone who completely gets it and who actually might derive something useful from it. My family and friends are humbling in their love and kindness but I don't want to bang on about it too much because they have their own feelings to deal with.

Please continue to share whatever you feel comfortable about.

With grateful thanks,

Sarah

Hey Sarah

Pleased to hear the last message was helpful! Thought it might be good to use some of the terms as they are... Can be helpful to know some so that you can be on a level with the doctors!

Sorry to hear you didn't get a result yesterday, it's tough when it drags on. Having you on dex for the time being seems sensible.

The mental gymnastics as you say can be very hard. It is a rollercoaster. What I understand with PBL, the relapses are a problem and this isn't great for mental health either! Treatments can be effective but then when you stop treatment  it can rebound. Like myeloma. They're learning how to deal with this Sarah. Given how few of us there are i think we are quite valuable and they will be learning from both of us. A longer term treatment of relatively low toxicity thalidamide and dex might be useful. My preliminary PET results are back. I have two areas of infection in my chest and prostate as expected. From a cancer perspective I have a suspicious lymph node attached to my bowel which they are going to remove/biopsy on Wednesday. This scan shows the result after 6 months on the above treatment. Previous to starting this, I had lymphoma lesions all over the place.

If it is the lymphoma that you are going to die from, yes that are many ways it could finally get you! But the same goes with every cancer and condition. You just can't predict the exact path it will take you down. It may or may not be as bad as you expect. But have comfort in knowing everything passes with time, nothing is permanent. 

The breath is very powerful. Listening to it, Focucing in on it. Must remind myself to do it more! That's good that you stayed fit through the last lot of chemo. I think I'd have been similar if it wasn't for the infections! Also good that you got to enjoy the remission time. Hopefully there'll be much more of this to come. Either after some more treatment or not if it isn't necessary.

Also grateful for the interaction. I know the benefit that people can get from speaking to like-minded people in a similar position. Support groups can be good for this. But I haven't found any other people with PBL through the main U.K charities so far!

All the very best for the results. Will speak again next week.

Best wishes

Simon

Hey Simon, 

How are you doing? How did your biopsy go? Presumably it'll be a few days before they get the results?

The thalidomide and dex sounds interesting. That has come up a few times in the research.

I got my biopsy results this week which confirmed that I have the same PBL as before. You may recall I had 6 cycles of EPOCH before. This time the plan is R-GDP, a couple of cycles and then re scan. The rationale is that this regimen is different chemo agents from EPOCH that are known to be effective in aggressive in non-Hodgkins lymphoma. They hope to do stem cell therapy. 

One step at a time. I start the treatment on Monday.

I am finding the waiting very challenging.  I can, for the most, part keep myself distracted and positive but it is mentally exhausting to do it! Knowing the tumours are growing while waiting for treatment is terrifying.

I do wonder how many of us there are in the UK just now. I think I read that statistically you have roughly a 1 in 2 million chance of getting this  (that's serious bad luck!) - which given the population of the UK means there are around 30-35 of us! No wonder it took so long for your radio signal to reach me! I often think of you posting the message on the forum and then waiting 5 months to get a reply! Had you nearly given up hope?? :)

I'll keep you posted on how the chemo progresses.

Best of luck with your biopsy result.

All the very best,

Sarah

Hey Sarah

I'm doing okay thanks. They couldn't successfully remove the lymph node- being guided by ultrasound so they are going to try again on Monday but being guided by CT instead. 

Sorry to hear your results came back positive. I know it'll be disappointing. I have not heard of R-GDP before. 2 cycles doesn't sound too intense.. Looking up a bit about it..it's partly immunotherapy- Rituximab? Does that mean your cancer cells are CD20 positive? Mine aren't unfortunately so Rituximab has never been an option for me. Have they also said if they'd like to do an autologous or allogenic transplant? Sorry I'm firing the questions!

Try not to worry about the wait for next week. They haven't left you cold turkey. The steroids actually have an anti cancer effect in themselves, so should be suppressing things until you start your regime. I'd try and make the most of this weekend if you can before you start treatment. I think the weather should be good! 
 

I know it's really is bad luck Sarah but it also makes us incredibly special :-p  haha. I had kind of given up hope to be honest. But I thought 'you never know!' And sure enough I got the message! Just to confirm I'm not medically trained in anyway Sarah so don't take anything I say as a completely accurate but I have picked up quite a lot along the way!

Let me know how you are getting on with your treatment and I'll keep you posted on the biopsy. But no pressure on the messaging. If you don't feel great on the treatment, just message when you can!

Keep your head up.

Simon 

Hey Simon,

Thanks for your mail!

I really hope your CT procedure went well yesterday and you are not feeling too sore today. When will you get your results?

Ah, I knew the cnacer was back so it. I've been feeling and watching it growing. It wasn't really a shock to get the results.

My chemo regimen: Yes, the consultant was checking that there wasn't a trace of CD20. The rituximab was always in doubt. So no it was just GDP yesterday. All went well,I felt a bit sick and tired by the end of the day but I reckon that was the dexamethasone. It really plays havoc with my blood sugar. Felt better after eating. The Consultant is keeping a close eye on the situation so I am seeing him towards the end of the first cycle just for him to see if here is any evidence it is working. If so, he'll do some imaging after 2 cycles. I am booked in for 4.

I've felt really well today. No nausea or fatigue. Been for a walk and did some stretching in the sushine. I'm under illusions that there may not be worse days ahead.... but I am very grateful for this day!

I think the intention is to do an autologous stem cell transplant. Was that what you were hoping to have? Have you seen much on the outcomes on that for PBL? I've seen individual case studies that look positive .... but that is generally all there is isn't it?- single case studies.

I appreciate your input and your questions Simon. I feel like a novice compared to you!

I took your advice and had a brilliant weekend! We bought bikes and cycled them out of the shop. We have just moved to new area where we have long riverside cycles and huge parks so hope I get some well times to make the most of that over the summer. It was joyous!

I hope you are able to enjoy the sunshine!

Are you able to work or are your infections making you feel too unwell?

Look forward to hearing your welcome voice out of the darkness again soon!

All the very best, 

Sarah