CTCL Sezary syndrome

I know a little about CTCL Sezary.  Would be happy to chat if it would help.  

Hi Anna

Thanks for your response. My dad was diagnosed about two years ago and since then has become progressively worse and for the last few weeks has been struggling terribly with his skin. He is currently undergoing photopheresis, bexaratine and interferon, but his skin seems to be getting worse now and his lymph nodes are back up so he is awaiting another scan to see what is going on.

His quality of life is really poor and we just don't know what to do, if anything. Its v hard to watch.

Hello Lisa

I know only too well how hard it must be for you to watch your dad suffering in this way.   My sister had Sezary syndrome.  Sadly she passed away last year.  But that doesn't mean your dad will.  

I'm guessing that your dad's condition started in the same way - with itching all over his body.   I know that is really hard to deal with.  Helen had many different ointments and lotions which were supposed to help but did very little.   As the illness progressed and the diagnosis became clearer she had many different treatments - UVB, PUVA, Psoralen, photopheresis, interferon, methotrexate, gemsar, doxil, romidepsin.   I know these names because with each new treatment I googled to find out as much as I could.

I don't know where your dad lives, but hopefully he lives close to a hospital which understands this very rare condition.  How old is he?

After about two years a very new treatment became available (Helen lived in the States, but I think it is now available here in the UK).   It is called Brentuximab.   Helen got permission from her insurers to embark on this treatment, which would hopefully prepare her for a stem cell transplant, for which I might have been a suitable donor.   She responded really well to the first two infusions on Brentuximab, but sadly she had become so frail following all she had been through that she was unable to continue.  Helen was 72.

I expect you have been looking up stuff on the web, and hopefully are finding support from Macmillan.   I also found the Lymphoma Association very helpful.   And in the States there is a wonderful organisation called the CTCL Foundation.  The CEO of this organisation, Susan Thornton, was a great help both to Helen (she talked to her on the phone) and also to me - I met her at a conference organised by the Lymphoma Association in the UK where she was a speaker.

I can't offer you an answer Lisa, but I do offer you and your dad my very best wishes, and I wish so much for him to pull through.   It must be so hard for him, and for you.   I do feel for you.   If you want to chat either on this, or on email, please feel free to do so.   anna_duckworth@Hotmail.com

Hi Ladies,  I have Sezary syndrome and posted the original questions on here last year. I was diagnosed in April 2014, I am now 56.  I'd be happy to join any discussion or provide as much information as I can if it will help.  My treatment is currently fortnightly photopheresis and daily Bexarotene.  I couldn't tolerate methotrexate & the bexarotene impacts my lipids so a roller coaster in sorting.  My sister is being tissue tested to see if she is a possible match in case stem cell transplant is the next option. 

I feel for you and your dad. IIt's a very waring lymphoma.  I often refer to myself as a human snow globe,  I shed so much skin (& now hair) it sometimes seems impossible to believe.   The house never stays clean,  as soon as it's done the skin is there again.  

How old is your dad Lisa?   Is he still working?  I decided to take early retirement and remove as many external stressor as I could and aside the lengthy process of tablets and creams, try to fill my days surrounded by fresh air (albeit covered up) a sense of humour & positive people. Not easy but it does help. 

Happy to continue talking here or if you prefer  please email me at alison_collyer@hotmail.co.uk. 

Meantime I wish your dad and you all the very best & I so hope he gets some relief. 

Thanks so much to you both for replying, its nice to speak to someone who has experience of Sezary as it's so rare.

My dad is 66 and so doesn't work now, he is very tired all the time and the rash now doesn't only itch it burns and his skin is extremely tight. He doesn't do much and can sleep most of the day some days, when he is awake he can fall asleep on the sofa at the drop of a hat. He was a very active man and now has such a poor quality of life. I have told him about the stem call testing, which was mentioned at the start of it all, about two years ago, but in a 'we'll try that if nothing works' way, which I think we are getting closer to now.

I can empathise with the tiredness and the burn. My daughter will visit and next thing I've nodded off! Does your dad have problems sleeping? I find the itch is worst at night and often keeps me awake and certainly disturbs my sleep, that doesn't help the day time tiredness.  For me the burn is like the itch, an intense feeling of something like sunburn but deep in the skin although my skin often feels cool to the touch.

I've tried many emollients and find it best to rotate them, it seems to help a bit. Emollient spray is excellent as you don't need to touch your skin, just spray it on and it's cooling, at least at the time.  If your dad's skin is really tight, it might be worth trying coconut oil. It's a solid form but disolves easily and certainly helped mine. If your dad doesn't  like the smell you can buy a deodorised one.

Re the tiredness, is your dad anaemic?  My meds and photopheresis have made me slightly anaemic so i very gone back to eating some red meat as well as the usual array of green leafy vegetables & that has elevated my HB & hermatecrit a little & again has helped with the tiredness a little.

Whereabouts are you Lisa?  Are you close to a hospital that has a good understanding of the condition?  Thankfully I am in Bristol and the consultants there do have some knowledge and experience treating sezary.

Hi Alison

My dad really struggles to sleep at night too, which is what he puts down to falling asleep so easily through the day. I'll let him know about the oil and anaemia for him to find more out about. My dad's in west yorkshire and they seem to have some knowledge of it, but not a lot with it being rare. It's difficult knowing so little.

Hi, I am new to this group as I do not seem to have the time to get as involved as I would like to. I was diagnosed in 1995, when I had a biopsy taken of a small dry skin irritation on my waist, with what I was told was a flare of 'T-cells', did not take much notice as they prescribed a topical ointment and the patch of dryness subsided. Then in 2011 on returning from our holiday abroad I had what the doctor assumed was a heat rash and prescribed accordingly, this did not seem right when there was no response to the treatment, I was referred to a specialist at the Dermatology dept at the NNUHosp where another biopsy confirmed a flare up of what they then said was Mycosis Fungoides!!!? No idea what it was until it was explained to me that this was a Non Hodgkins Lymphoma condition, CTCL (Cutaeneous T-Cell Lymphoma)(Skin Cancer) and sent me of to have Phototherapy (sun lamp) treatment, this did not respond well over 3-4 months of treatment and I was then referred to Haematology/Oncology where they determined my Lymphoma condition had spread to my blood, diagnosed as Sezary Syndrome, whereupon I was referred to have treatment in London at St Thomas's Hospital with the process of Photopherisis treatment, which began in Jan 2012. In 2014 it was decided that things were not quite progressing well enough and decided to prescribe Interferon Alpha injections to improve stabilisation of the skin conditions, and I am still following these courses of treatment to date but now I am doing this in the new departments in Guys Hosp London. Eight weeks ago I was subjected to a one off radiotherapy treatment to my left ear to try and reduce the tumours in my ear lobes and skin tags behind the ear and will be seeing my consultant in November as to how things are going.

What I am trying to convey to you is that, I try to keep a positive attitude to all of these things we have to deal with and take them in my stride, yes I get tired and sometimes irritable but I put these behind me  by not giving in. I try to lead a healthy balanced lifestyle with nutrition(not exercise) and activities,getting out as much as we can afford. When I need to do things I pace myself and try not to do too much in one go then things get done and I feel I am coping well. My wife and family are the ones I feel sorry for as they worry so much about how I am and what the future may hold. I try not to look too far ahead but try to enjoy life of the now. I hope this may be I some help to you

Hi Anna and Lisa,

Anna, I'm sorry for your loss and what your sister and you and your family endured.  Lisa, I hope your dad finds relief from his condition soon and I understand what you're going through.

My mom, who lives in the U.S. (in Connecticut) and is 73 years old was diagnosed with Sezary Syndrome just over a month ago after a year of misdiagnoses including eczema and Pityriasis rubra pilaris (PRP).

During the time of her misdiagnosis, she was treated with various creams, steriods, retinoids, etc.  One of her treatments then was methotraxate, which I understand now is sometimes used as a first-line treatment for Sezary.  None of those treatments really helped her condition and during some of those treatments (particularly with Acitretin) her skin "peeled in sheets" as she described it.

When she was diagnosed in September of this year with Sezary Syndrome, the doctor started her right away on two back-to-back days (2-1/2 hours per day) of Photopheresis (ECP) and  three days later on Targretin (Bexarotene) at 1 pill per day (since her body reacted so strongly to the Acitretin which was also a retinoid, he started her on only 1 pill per day vs. the typical 5 or 6 pills per day).  She had a major flare up of the skin after the third day of Targretin and was in excruciating pain and described her skin as "crispy crispy".  On a scale of 1 to 10 for pain she said she was well above 10.

The next day she saw an oncologist at Yale who took her off the photopheresis and Targretin.  Mom started taking OxyCodone 5mg 2x a day to manage the pain which helped tremendously.

The doctor said that based on mom's flow cytometry test results the Sezary was very aggresive and comprised 66% of her white blood cells so a more aggressive treatment was needed.  She was going to recommend that mom start on a regiment of Romidepsin (Istodax) but then I asked her about a Phase 3 Clinical Trial currently going on for a drug called Mogamulizumab (also known as KW-0761), a  monoclonal antibody that targets CCR4 receptors, which is approved for use in Japan but not yet in the U.S.  It is being tested against Vorinostat (brand name is Zolinza), a Histone deacetylase inhibitor that has been approved for use in the U.S many years ago. 

My mom entered the trial and was "randomized" to the Vorinostat.  She is on that for 8 weeks and we'll see if it helps her condition.  My understanding from research is that about 30% of Sezary patients obtain a least a partial (50% or better) response using Vorinostat.  If her body does not respond to the Vorinostat, then the clinical trial allows her to switch over to the Mogamulizumab.  My understanding is that about 47% of Sezary patients have had a least a partial response using that treatment in Phase 1 and Phase 2 trials.

I think the trial is going on in a number of hospitals internationally (not sure about the U.K.) and might still be accepting new patients but if so, maybe not for long.  I heard it would be closing to new patients soon so it might be worth asking about.

I know of Sezary patient in the U.S. who has just started on a low-dose subcutaneous treatment using Alemtuzumab (brand name Campath) based on the results in this study:  http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4092049/  The patient is the mom of someone I am connected with on Facebook; he has done a lot of research on treatment methods for Sezary.

Feel free to contact me at mikegrayeb@gmail.com if you'd like.

Anna, if it's ok with you may I reach out to you at some point?  Thank you for your consideration.

I wish you all the best.

Sincerely,

Mike

In my desparate search for answers I suddenly reached this website. Since I am from the Netherlands English is not my native language, so I apologize in advance.

After 3 1/2 years my mother finally seems to be diagnosed with Sezary. No one ever talked about mycosis fungoides and related terminology, they only seemed not to be able to choose between eczema and psoriasis, until three months ago, then Sezary was named. My mother's skin is almost 100% red and very very itchy, from under her skin as she describes it. The only medicine that give some relief is prednison; she just had 2 times a week for 10 weeks puva-light therapy without any recovery or improvement. Now the radiologist will have to decide whether radiation therapy is necessary or advisory. At this moment any information is welcome, because here in the Netherlands it is extremely difficult to find any relevant information to this disease let alone contact with people or relatives with the same diagnosis.

I would like to thank you all in advance for just reading my post and by doing so giving in that way the feeling that we are not alone in this terrible fight.

Imsk