Treatment Options for Peripheral T-Cell Lymphoma

The Leukemia & Lymphoma Society is doing another free web/telephone education program, this one on PTCLs, taking place at 1 Eastern March 23. It's easy to sign up and easy to participate. Click on the link below for more details.

Treatment Options for Peripheral T-Cell Lymphoma

Thanks so much for sharing this information with us Ross!

Thanks Ross but I already sign up...

thanks- i am new to the site and wanted to know what treatments you are/were on for PTCL. i have tried 2 rounds of chop and one of ice and they dont seem to work. i am now going to try pralatrexate next week.

also can't sleep but for more than an hour at a time; it is all so exhausting.

Courde, I would like to welcome you to Support Groups. I am hoping that one of our wonderful Supporters will give you some insight and information. You are in my thoughts and prayers.

Courde,

Unfortunately, CHOP has been found, as you have discovered, to be ineffective against PTCL. As well, ICE was offered to me only as salvage therapy. PTCL is of such a rarity that there is not standardized treatment for it. Thus, the patient is at the mercy of the doctor's knowledge and judgment. Do you have a second opinion from an organization with substantial PTCL experience? This can be crucial to obtaining effective treatment.

My first (general) oncologist believed that I had a virus and wanted to wait. Had I done that, I would not be writing this. I went from a local clinic to the Seattle Cancer Care Alliance/Fred Hutchinson (www.seattlecca.org/). There, I was properly diagnosed and was administered eight cycles of CHOEP (EPOCH) and eight cycles of GVD. The strategy was to use two completely different mechanisms in treatment, first weakening the cancer by attacking from one direction, then switching direction and attacking via a different mechanism, in hope of defeating it. This reasoning was used since the cancer has the propensity to mutate around a single type of treatment (even though multiple agents are used). I obtained full response from the CHOEP-GVD regimen. However, the cancer relapsed immediately post-chemo, which was not unexpected - but made a poor prognosis even worse.

I was then staring at a 24 hour in-patient infusion of ICE, which offered no real hope, since I had already received Etopiside, one of ICE's components. At that time, I was offered a clinical trial of Romidepsin (aka: FK228, Depsipeptide, Istodax) as a single agent. It is biological and not a chemotherapy drug. I agreed to try it, with the proviso that ICe could still be administered if the Romidepsin was ineffective. My variety of PTCL-NOS did not like the Romidepsin at all and went immediately into full response. Median time of response to Romidepsin is 13 months, but I am still responding past 25 months. Romidepsin's toxicity is far less than chemo and the other patients and I are providing long-term side effect feedback. It is FDA approved for Cutaneous T-Cell and for B-cell lymphomas which have relapsed.

There are various treatments now that did not exist in the fairly recent past. However, since I am post-chemo, most of the programs I am aware of are targeted at relapsed disease. I am approaching the three-year mark, so hope is definitely on the rise. Just take charge and be your won advocate. No one else speaks for you as well as you do.

All the best.

Jim

Hi, Courde! Welcome. We're glad you found this group.

I've taken Prednisone at high doses for weeks and that made it hard for me to sleep.
I've read that soneone had asked their dr. if they could take a little benedryl before going to bed at night. I always have a list of questions to ask the dr. and bring pen & paper with me all the time.

Ross, thanks for sharing all the info you do.
We really appreciate it.

Hi Jim,

Thanks for your message. I am currently on Romidepsin and my fourth treatment will be on Friday, April 15th and a pet scan the week after. While on Romidepsin, the drs and I have seen improvement with the edema, the look and feel of the skin as well as my persistent cough; I am able to better and also have a conversation with almost no shortness of breath.

I noticed that I am having small nodule growths- 3 on my left side and one on my right as of Sunday, April 10th. One of the nodules has become a deep red color and we have contacted the dr and will meet with him today.

If Romidepsin continues to perform the way it has, we can start discussing bone marrow transplant options at Vanderbilt in Nashville. God willing I will have the same success you have had with the Romidepsin.

courde,

Glad to hear that your doctor has made this drug available to you. Even though it may be considered cutting edge, there are others arriving. My doctor asked me to speak with another PTCL patient whose disease had relapsed immediately post-chemo, as mine did. She has been offered Romidepsin as well as Belinostat (and Pralatrexate if I remember), which is also aimed at relapsed lymphomas. I have looked into Belinostat, as I might have need of in the future. But, I see that Belinostat is also an HDAC inhibitor, like Romidepsin, so it may not be effective following another HDAC inhibitor. The good news is that those cancers that do not like Romidepsin seem to really dislike it, so all of the best to you.

I will include you in my prayers. On the spiritual side of things, I found that once I simply abandoned myself to God's Divine providence, and released any "hold" that I thought I had on this life, the whole struggle became easier, and He handed my life back to me. I know not the status of your faith life, but delving more deeply into it can bring amazing comfort and strength.

Jim

Hi Jim,

My dad went to the drs today and the nodules are the lymphoma, not an infection. More have become apparent in the past four days showing that Romidepsin isn't working as well as the drs had hoped. He will be starting Pralatrexate today. Have you heard of positive reactions to this medicine?

Thank you for your support, its been wonderful hearing from people and families who have had or currently going through this.

Best,
AG

Here's the Wiki: http://en.wikipedia.org/wiki/Pralatrexate

It received FDA approval slightly before Romidepsin did. What we know is that "NOS" is a broad category of PTCL that requires a wide range of anti-cancer agents to address. I see that Belinostat is also an HDAC Inhibitor, just as Romidepsin is, so that may not be an option for either of us. Since Pralatrexate uses a different mechanism against the cells than Romidepsin, chances for success probably increase.

I see that my oncologist was a member of this study: http://jco.ascopubs.org/content/29/9/1182.abstract

The FDA saw a positive response rate that qualified Pralatrexate for "fast track" approval.

Hi Jim,

Thank you- I got the article from Oncology Stat as well which was helpful. Let me know if you would like me to send your way. My dad's oncologist is the editor for Oncology Stat so I hope he is well read on all of these medicines/treatments.

Were you at a specialized center for PTCL? My dad is currently going to a clinic.

What worries me is that Romidepsin was working so well for the first 3 sessions and then all of a sudden all these nodules came up. My mom said that he has around 20 or so all over. Romidepsin reduced his cough tremendously as well as showed major improvement overall and it was so sudden that we had to stop it. I sometimes wonder was it too quickly done? Were you seeing improvment after each week or did you ever have a set back with the medicine?

We will try to Pralatrexate and if not, Belinostat it is. Also, did you ever consider a stem cell transplant?

Thanks for all your help, this is really helpful to discuss.

Best,
AG

I am a patient at Seattle Cancer Care Alliance (SCCA, www.Seattle CCA.org/), the treatment arm of Fred Hutchinson Cancer Research Center. The problem with PTCL, for most patients and even for most oncologists, is that they may never have encountered or treated it before. That was the problem with my first (non-SCCA) general oncologist, who thought that I had a virus and wanted to wait. If I had waited, I would not be here. My wife was my angel here, scheduling the consult at SCCA for a second opinion. My oncologist, Dr Andrei Shustov, was involved not only in Romidepsin trials, but also Pralatrexate and the newer Belinostat. I can recommend him with absolute confidence, as his judgment in my case made the difference between surviving or not.

A second opinion, from an oncologist at a major treatment center is crucial to experiencing a good outcome in PTCL, because of its rarity as well as its virulence. But, we must not be afraid of it, but rather face it down, while loving the life that we have been blessed with.

Your dad's problem is common to PTCL sufferers, in that the disease either: was not eliminated by common types of chemotherapy (i.e. CHOP), or it adapted to and mutated around the other therapies that it encountered, including novel treatments. This is the concern that dictated my primary treatment, which involved eight different anti-cancer drugs. The regimen was quite effective in either eliminating the PTCL completely, or weakening it to the point of non-detectability.

I can imagine, at some point, seeing a protocol involving a revolving combination of novel therapies, involving Romidepsin/Belinostat, Pralatrexate (Folotyn) and other novel drugs as they become available. One developing theory is to change strategies during treatment and attack the disease using an entirely different mechanism - a sort of "luring" the cancer to mutate against a certain drug, then suddenly changing drugs against it. This worked in my case, even though I immediately relapsed post-chemo. What we are gaining from all of this is a knowledge base from which medical science can develop some form of standard treatment in PTCL cases.

In my case, after I began Romidepsin, the disease responded immediately until no further evidence of it was detectable. Of course, it was only at stage I when secondary treatment was begun after relapse. I have had no sign of disease for two years now. Median time of disease response to Romidepsin is 13 months, whereas I am now in my 26th month of response. I have been reduced from three cycles monthly to two. Once the protocol is re-written, I expect to go with a single monthly treatment, which will be a blessing.

As to a transplant, my blood stem cells are in cryogenic storage at SCCA in case I need an autologous transplant. However, the statistics indicating that those who respond to Romidepsin (and other novel therapies) receive the same benefit as transplant patients, but without the substantial risks (and costs) involved in a transplant. I am limited to my own harvested cells, since there is no known match in the world book of marrow donors, and it is not likely that one will ever be found. Of note is that my blood stem cells revealed no traces of disease 8 months past the institution of Romidepsin therapy. Doctor was first planning on a transplant, but no longer is considering one, due to my response to the Romidepsin. As well, an autologous transplant will only restore the same immune system which failed and allowed my PTCL to develop in the first place.

However, my case is anecdotal in nature, since there simply is insufficient data to construct a complete disease response profile. The picture is beginning to take shape, but is far from clear at this point.

As I touched on above, my advice is to get second opinions and, if possible, travel to a globally known treatment facility to avail yourself of the greatest knowledge base and treatment options. And pray. My life hangs by a thread called prayer.

Jim

Hi Jim,

We have all my dad's files scanned- do you think that Dr. Shustov would be able to give us a third opinion or if he knows someone in Tennesse (or anywhere else really) we could speak to them??

My father is unfortunately in no condition to travel from TN to Seattle and though we have been working with a specialist in Nasvhille and an increadible oncologist in Memphis, I am willing to ask anyone who may have something that will work on my dad.

He got his first treatment of Pralatexrate on Monday, April 18th and we had a CT scan on Thursday, April 21st. The scan showed that my dads lungs had cleared up (i believe this was because of the Romidepsin- my dads cough had also improved which allowed him to sleep for a solid amount of time at night). Since Monday, April 18th my dad has developed about 15-20 more nodules, mostly on his face, which totals to over 40 now and now the cough has also been coming back slowly.

I just spoke to him and he can't really use his hands because of the swelling so my mother has to feed him etc.. Also, he hasn't experienced any of the chemo side-effects such as nausea or blisters from Pralatexrate (he hadn't had any of the chemo side effects with CHOP or ICE either, only Romidepsin) which makes me wonder if the cancer is being affected by pralatexrate. Is that something you noticed within your own treatments? Also, is one chemotherapy too soon to be getting side effects?

I would imagine that combining chemotherapies would "trick" the disease, but I don't know if that is possible to do. Is that what they did in your situation, I wasn't able to follow that completely..

We are going to start researching clinical trials and treatments abroad as a family to know what options we have. Dad has his second does of Pralatrexate tomorrow and I just hope that we start seeing results.

Thanks Jim, looking forward to hearing from you soon.

Best,
AG

AG,

What is your dad's variety of PTCL? From what you describe, it sounds like it is manifesting itself in his skin, which mine did not. Mine was confined to the lymph nodes and the bone marrow, but it may show up in other organs, such as the skin. There is also CTCL (Cutaneous T-Cell Lymphoma) which manifests itself mainly in the skin, but other varieties also present themselves in the skin, which may explain the lesions that your dad has..

I would not hesitate to consult with either Dr. Shustov, one of the other lymphoma specialists at SCCA, or with his counterpart at Sloan-Kettering, for example. Consider that, prior to working at SCCA, Dr. Shustov's nurse spent 15 years in oncology and never saw a case of PTCL, due to its rarity. That is why additional opinions are crucial.

As to the side-effects, they may be evident at different times in different patients. Not all Romidepsin patients experience the side effects that I do, for example. There are common side-effects associated with each anti-cancer drug, but some patients may not experience those exact effects, or may experience a different set of effects at a different time.

Unfortunately,, there is a fairly broad spectrum of similar, but not identical, diseases within the category of PTCL, with the largest group being labelled NOS, or "Not Otherwise Specified". Thus, they respond to differing therapies. The cancer remains so rare that there is no standard treatment for it. The fact that mine responded well to the CHOEP/EPOCH-GVD therapy (and subsequent Romidepsin) does not indicate that anyone else's PTCL would. The oncologist's experience here is very important, as are sophisticated biopsy test results.

Time is of the essence, and you may very well be your dad's best advocate. Just keep pressing until you exhaust all reasonable resources.

My prayers are with you.

Jim

Thanks, Jim.

My dad has PTCL-NOS and unfortunately the skin and lungs are affected. Though we have seen improvement in the lungs, I know time is of the essence and will reach out to everyone and anyone I can. I just want to make sure we are trying everything.

I know that every individual and case is different but I have to admit, when I read that Romidepsin worked so well for you and it was working well for my dad, I became very hopeful.

I will reach out to Dr. Shustov and whomever else he suggests. I am hoping calling is the best method and I can email him whatever he wants.

Thanks for your support and prayers. We will be in touch.

Best,
AG

Hi Jim,

How did your family deal with your cancer? My brother and I are both in our mid-20's so its definitely been difficult for us. What is harder is we aren't with my dad all the time so have weekends with our parents...

Any advice would be great. It is hard to just keep faith and positive all the time...esp. on the days you get negative news...

Best,
AG

My family drew closer together and relied much more on their faith than they did before. Belief in God seems a very nice thing, but it is something we generally keep at a bit of a distance. When news such as this strikes, it is time to draw near to what you believe. If you have a faith life, now is the time to dive into it. Our lives are extremely fragile and hang by a thread which we cannot see. In my life, that thread is called prayer. I have zero doubt that the reason I am typing this to you is through the prayers of many who lift me up.

There is nothing of this world which will completely satisfy us. Everthing falls short at some point. Medicine offers hope, but it is a limited hope. Therefore something , or some One, must make up the difference. Some one bridges the gap, extending a hand and lending us strength. Time to be bold. Ask those of faith to pray for your dad and for your family. It makes a huge difference. The further we are able to see beyond this life, the greater our hope is. I have hope, both in this life and in the next, because I made an act of the will to believe. And, that is all that hope costs - an act of human will. And, your will is yours. No one can take it from you.

We live in a world of darkness - one of doom and gloom. Hope is the point of light that grows only brighter as we approach it. I can counsel you only to make an act of the will and move toward that hope.

Prayers for your family.

Jim

Thanks Jim.

I contacted Dr. Shustov today and we e-mailed him my father's reports and a summary. Very kind of him to review; I mentioned that I was speaking to a Jim who is a patient of his and he seemed to recognize who you are..

Our Dr. in Memphis decided Pralatrexate did not seem to work after only two rounds. I am confused and frustrated because the research says people dont see reactions until after 4-6 rounds.

This leaves us to our last option GVD tomorrow. We did resend the path to the NIH to see if we can find more markers.

Will keep you posted...thank you for your support.

AG,

Your dad is being treated for the first occurrence of PTCL, correct? If so, then the HDAC inhibitors that I mentioned may not be the optimum just yet, as they are targeted at relapsed or refractory disease. The GVD (8 cycles) worked well in my case, but it immediately followed eight cycles of CHOEP (EPOCH). It is a tough, aggressive course and when it is approaching the final cycles, it does take its toll on the human body. The effects seem to hit men harder than women - at least one aspect of the toxicity. But, I survived it at age 56, and I'll be 59 next month, thank God!

Dr. Shustov is so busy that I am sometimes surprised that he finds time for clinic at all. However, I have never doubted his skill or judgment throughout this journey. Cancer is the enemy and he is dedicated to eradicating it.

All the best,

Jim