T-PLL or AITL - looking for treatment advice please

[ingeanette]

Hi there,

I am new here but looking for treatment advice. My mother is diagnosed with either T-PLL or AITL, FISH showing p17 in 2/3 of cells.

She has undergone 3 x Campath treatment with good results but now struggling with higher wbc and very swollen lymphs, mostly in legs/feet. It s causing pain and due to very dry skin also starting to crack her skin under feet and on fingers.

Just started Vidaza (azacitidin) given 5 x weekly as injection under the skin followed by 3 weeks off: no effect as such yet but guess its also early days. However, I am wondering if anyone with T-PLL has some advice to share as the T-PLL is so rare? I read about venetoclax with great interest.

Thanking you in advance and wishing all the very best.

[Panz]

Welcome to our community and this is a great place to be....I am so happy you Mom has you...I just love the caregivers of the world...they are such special people!!!

I have no knowledge about what you are asking but trust there are those in our community who do or are able to direct you in the right direction. ☘️😍🙏💕

[ingeanette in reply to Panz]

Thank you for the kind words Panz.

I am hoping to hear what treatment possibilities patients with T-PLL or AITL have had bearing the p17 in mind. Has anyone with p17 had experience with Venetoclax? Perhaps also after having received the Campath beforehand. And any advice for dealing with the swelling? Twice a day we try to do a foot bath with white soap and then apply a fat creme but any suggestions welcome

[Cllcanada Top Poster CURE Hero]

Hi and welcome

I'm sorry to hear about your Mum... T-PLL and or AITL is a T cell lymphoma, where CLL and B-PLL are B cell lymphomas, they are apples and oranges.

Venetoclax is drug targeted to BCL2 in the B cells, do you know if she is BCL2 positive?

There is a clinical trial underway using venetoclax.. you can find it here. Unfortunately it is in Italy.

clinicaltrials.gov/ct2/resu...

Campath is the standard treatment, but drug cocktails are often used.

Here is a good overview of AITK and it discusses treatment options

lymphoma.org/wp-content/upl...

Also peripheral

lymphoma.org/wp-content/upl...

I hope you can get your mother to a major research hospital, they may have greater insight into treatment of this rare disease.

Please note that much of what you read on this CLL forum won't apply, but you are welcome to stay, should you wish.

There are a few members here with B-PLL, but again its apples and oranges...

I hope you can get some answers.

~chris 🇨🇦

[ingeanette in reply to Cllcanada]

Cheers Chris - thank you so much for your kind advice.

It has proven quite difficult to obtain info about the T-PLL and since the Campath was also mainly directed for B-CLL patients, I thought it would make sense to try to investigate further into the possibility of other shared/common drugs. I was reading an article in medicalxpress.com about a research group from MedUni Vienna last year who found out that T-PLL responds particular well to the drug Venetolax. I need to ask my mothers doctor about the protein BCL-2 as you rightly said to see if it s a possibility for her.

Hope it could be helpful for others to know too.

[LAinNYC]

Hello, my husband has CLL, and took 13 injections of Campath when he had a rare anemia, called PRCA. It got him a 2 1/2 year remission, not without complications, and he recently started on Venetoclax following 8 doses of Rituximab. He's not yet back in remission, but doing well. A specialist is vital, since everyone's disease course is unique. I hope this helps.

[ingeanette]

Very helpful - thank you so much for sharing.

My Mother finally today told me that her swelling it a tiny bit better, so hopefully the Vidaza is starting to work (she has had 2 rounds of 5 days each). Thank you again.

[T-Pll-UK]

Hi, I have T-PLL, I was diagnosed in May this year.i was 100% symptomatic and was started in campath. Tomorrow I will have my 36th campath infusion. I have been infused 3 x a week and it’s brought down my wbc from 130 to 1.9. I hope to have a stem cell transplant in November. I have found the T-PLL Facebook group a huge source of support, as this is such a rare and nasty disease and there aren’t that many people with it. With all the different health systems around the world options for treatment and available drug differ however there are world renown medical specialists in that group with cutting edge knowledge over developments. I hope your mum is responding to the treatment she is receiving. All the best.

[ingeanette]

Hi T-PII UK - I have jost joined the Facebook Group so thank you so much for mentioning that option. My Mother is still having the Vidaza treatment once a month and although she is a bit more tired than usual and have lost a bit of weight, she has luckily no longer swollen lymphs nodes in the legs/feet. Her skin rash is also much better after having had Electron Radiance 15 days in a row - all the dryness has improved immensely.

Wishing you best of luck with the stem cell transplant in November, we also looked into that possibility but unfortunately we could not find a suitable doner match. All the best.

[T-Pll-UK]

Show your medic this and tell him/her another paper is due out soon.

bloodjournal.org/content/12...