TPLL

[Jince45]

Is there ever anything posted on this site or in Health Unlocked about t Cell prolymphocytic leukemia? My husband passed away in December of this disease and I am interested in any new developments or treatments.

[Psmithuk]

I'm so sorry to hear this, jince45. I have no knowledge of this disease, I just wanted to send you my sympathy, and hope you are OK.

It might be worth 'locking' your post, to improve your chance of getting a reply.

[Jm954 Administrator]

Hi jince45, so sorry to hear about your husband. He was very unlucky to get T cell PLL, it's extremely rare.

It's very different to CLL, which is what this site is devoted to, as it's a leukaemia of T lymphocyte cells.

T PLL is very difficult to treat and, because there are few patients, it's difficult to get significant patient numbers for trials of new treatments. Treatment has mostly been monoclonal antibodies (Campath) followed by transplant but there is a newer treatment called Brentuximab Vendotin which is an antibody/drug combination. It attaches to the CD30 on the T cell and delivers the chemo directly to the cell. Nelarabine is sometimes used but I don't know anything about this, it's usually used for acute leukaemia.

Despite treatment and transplant T PLL remains incurable with these treatments and sadly many patients do not have a very long remission, usually only a few months.

Sending best wishes.

[Jince45 in reply to Jm954]

He was treated with Campath for 5 months but it did not work. Our oncologist also tried Ibrutinib as a last resort but it also did not work. He passed away 6 months after starting to show symptoms as is the life expectancy of patients with this disease as written in all of the articles that I have read.

[Jm954 Administrator in reply to Jince45]

T PLL is typically very aggressive and I'm so sorry to hear Campath didn't work for your husband. Sending my sympathy, take care x

[wroxham-gb]

I would just like to send my best wishes to you and I am so sorry you have recently lost your husband.

Best wishes.

Sue

[pkpayne]

Hi Jince45, I don't have any information about the disease you inquired about but I wanted to pass along my deepest sympathies for your loss. I'm sure someone will come along soon that might be able to help you.

[marcyh]

Jince45 - I am so very sorry to hear this. I know of someone with T cell PLL and will see if they would be interested in corresponding.

[LuluMcManus]

Hi Jince,

I am so sorry for your loss. My father in law was just diagnosed with TPLL. He was previously diagnosed with CLL but as of today his oncologist is now saying TPLL. I wasn't at the appointment, so I didnt hear all of the details, but of course my husband and I are now researching everything online and it's grim. I know it must be so hard on you, and I'm sorry to pry, but I am wondering if you would be able to tell me if your husband had symptoms when he was diagnosed? My father in law is the type to never let us know if he was really having symptoms or not. We have an appt on the 20th to discuss starting Campath.. but I'm starting to wonder if we should. Was it hard on your husband?

Once again, so sorry for your loss. Cancer SUCKS.

[Cllcanada Top Poster CURE Hero in reply to LuluMcManus]

Hi Lulu

I find it a bit odd they would confuse CLL a B cell lymphoma with T cell PLL.

However, there is a history of T-CLL, but it was reclassified as T-PLL 15 years ago. CLL is now exclusively B cell.

You might wish to talk to directly with your father in laws doctor...

The immunophenotype is quite different from CLL

T-PLL has the immunophenotype of a mature (post-thymic) T-lymphocyte, and the neoplastic cells are typically positive for pan-T antigens CD2, CD3, and CD7 and negative for TdT and CD1a. The immunophenotype CD4+/CD8- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases.[2]

en.m.wikipedia.org/wiki/T-c...

Diagnosis of chronic lymphocytic leukemia (chronic lymphoid leukemia, CLL). It confirms the presence of circulating clonal B-lymphocytes expressing CD5, CD19, CD20(dim), CD 23, and an absence of FMC-7 staining.

~chris

[Jince45 in reply to Cllcanada]

I also find this hard to understand that the 2 have been confused.

[LuluMcManus in reply to Jince45]

My father in law was first diagnosed 6 years ago because his routine blood work was "off." It took them about a year to figure out that it was a form of leukemia. Since his blood results kept changed, but very slowly, the first oncologist diagnosed it as CLL. Within the last year, his blood work started to change much more drastically to the point that the original oncologist began talking about starting chemo. When this news came, a few months ago, my inlaws decided to go to a different oncologist for a second opinion. This new doctor looked at his records and realized they hadn't done a full body cat scan, a bone marrow test, among other tests since his first diagnosis almost 6 years ago. So this new doctor ran every test under the sun and found out that his lymphnodes are slightly enlarged as well as his spleen. His platelets are low and his white blood cell count is 99.9. Off the charts. So with everything combined this new doctor has now said it's TPLL.

I don't understand how it all works and how different diagnosis can happen, but maybe it was TPLL all along but it just now is getting bad?

[Jince45 in reply to LuluMcManus]

Lulu

We were very fortunate to have a very astute family doctor and a great Oncologist/hematologist. All the tests and scans were done initially for my husband then he had regular CT scans throughout his illness. He developed clots in both lower legs and went into kidney failure. His spleen and abdominal lymph nodes were enlarged. He had a lot of side effects from the chemo.

I would be surprised if your father in law has had TPLL all along as the survival rate without treatment or in my husbands case with treatment is about 7 months.

I expect it may have been easier for us to understand as I have a nursing background and our son is a paramedic.

I was able to keep my husband at home until the last day. We moved him to the Hospice at 3 o'clock and he passed away at 8 o'clock that night.

I am not sure where your father in law lives but I live in Canada.

[Jince45 in reply to LuluMcManus]

Hi Lulu

My husband was diagnosed quite by accident in September of 2015. He had gone to his doctor about a nagging pain in his right side which was not related to his disease. His blood profile at that time showed he had TPLL. We did watch and wait with monthly blood work until July of 2016 when he began to show symptoms. He was tired, had lost weight, and his blood work started to change. We began treatment with Campath, 3 times per week on Aug 8th of 2016. In Sept our oncologist told us it was not working and began to supplement it with Fludarabine which also did not work. in October we started on the oral chemo Ibrutinib as a last resort. This also did not work.

The chemo was very hard on him. He experienced every side effect ever documented. He was a big strapping 69 year old who was very fit and young for his age and he never complained. The hope was to eventually get him to transplant but he passed away on Dec 13th 2016 which was his 70th birthday,

I live in Canada but have been in contact with both the Canadian Cancer Society and the American Leukemia and Lymphoma Association and the information i have received is not optimistic. It is very rare and I know there are oncologists here in Canada who have been working for 40 years and have never met a patient with this diagnosis.

I don't know the age of your father in law but I know from our experience that we would go through the treatment all over again if we thought there was even the chance of a few more months together.

[LuluMcManus in reply to Jince45]

Hi Jince45,

Thank you for your quick response. My father in law found out he had CLL, now TPLL, in a routine check up. His blood levels were off so they began monitoring him. The initial blood check up that showed abnormal white blood cell counts was almost 5 years ago. His levels have been changing ever since, but as of recent have began changing in a more drastic manner. His lymphnodes now show slight swelling, as well as his spleen. He doesn't feel bad yet and still has no physical symptoms. He is 70 years young. In great shape- was a firefighter for 30 years.

His latest blood results show a decline in his platelet count. He is now about 20 points under the lowest acceptable count. His white blood cells are off the charts high. His doctor says he wants to just keep checking his blood every few weeks and if his platelet count keeps going down he will need to start Campath asap. It sounds very similar to your husband as far as this doctor's "plan" for him. My husband and his brother as well as my father in law's siblings will be getting tested as possible donors. Hoping we get to that point. Praying.

Anyways, thank you for your input. I know how hard this is. And we are only just beginning. Obviously the decision will be my father in laws, but I'm just starting to wonder IF the Campath doesn't work... do you want to go through the chemo? Is it worth it? Of course we will want to do anything on this planet to save him- I just dont want him to go through hell and back for a very slim chance of it working.

Will keep you posted. He goes back for blood work on the 13th and then on the 20th I believe we will be making some decisions.

Hope you are well.

[KaPam]

I have TPLL. Yes rare but seems to be surfacing more lately?? I was diagnosed 3.5 years ago on routine blood work. Was treated with Campath and achieve remission. Almost 1 year ago I had a stem cell transplant from my sister. So far...labs and tests are normal. Wishing you all well and not throw in the towel. I know of a 17 year survivor and a 7 year survived. Anything is possible. Kathi