TPLL

[Whatadayitwas]

I want to thank you all again for welcoming me into your community. Mike's cancer diagnosis has been very unique. As has his treatment. I don't recall what I have already shared, but his actual diagnosis ended up being TPLL (Tcell Prolymphocytic Leukemia). His WBC count went from 432,000 on 9/30 to 530,000 on 10/2. He was admitted in 10/9 as soon as his meds arrived from the UK, his WBC was 535,000.

He is currently on Campath. He had to do a ramp up over 7 days 3mg on first day and then 3, 10, 10, 30, a one day break, and then 30. The drug is amazing and he is responding. The downside is that it explodes his Tcells at such an unbelievable rate that he has a risk of tumor lysis, hence the inpatient for treatment. The life of the drug in his system is 288 hours, so as the drug compounded in his system, so did the slaughter of the cells. His 2nd night of treatment he went into respiratory distress, hypotension, etc. They have to do what I refer to as deploying COUNTERMEASURES. This includes pretreatment for an hour an hour and a half with benadryl a, zofran and solumedrol. Once he starts having a reaction they have to stop the infusion and push allopurinol, rasburicase, and more steroids to prevent the hypercalcemia, hyperkalemia, hyperuricemia, and hyperphosphatemia. Luckily he has had great nurses and was watched carefully. Any time they had to stop the infusion, it was at the very end of the 2 hour drip. He would start with chills and immediately go into rigors. It has been difficult for him and scary, especially the first reaction. The first 30mg dose he was given resulted in a 50 minute episode of rigors, hypertension, and tachycardia. But he made it through. We have infusions 3times a week for 12 weeks at 30mg each.

His body's response to this treatment has been unbelievable. His spleen on CT was measured at 23cm in length and 23cm diameter. It is now slightly below his ribcage. His white count as of Friday 10/23 was 10,670. That is down from 535,000 two weeks earlier. The night of his 50 minute reaction, his WBC count went down by 170,000.

We still just don't know enough about this disease, and most doctors don't see a case of this more than once in 5-10 years, if ever. The average age at diagnosis is 67-70. And most can't survive the treatment, are misdiagnosed, or choose not to treat given their age. It looks as though we will be heading to University of nebraska to discuss a stem cell transplant. The leading expert on this disease is Dr Julie Vose at University of Nebraska and she is consulting with our hematologist regarding his case and treatment.

[Shedman]

Thanks for this update.

Good to hear that his treatment progresses fairly well.

I can picture his white cell change graph.. thought my own was ‘exciting’, going 270,000 down to ~16,000 in no time at all .. his of T-cells is ‘twice as exciting’ — a roller-coaster of a graph.

Fingers-crossed this treatment concludes well, and enables the next options as indicated/necessary.

I don’t know how a supportive partner alters the equation, lacking such; much of my time involved weariness and confusion, with rest being the main need.. However, I could have done with a close watch a few times when I developed a fever and still more confusion - my out-patient journey was more of luck than my good judgement, happily the medical staff were more competent than me.

Good luck with all the emotions and worries.

[Slowmo457]

Hoping for Great success with his treatment. Best Wishes 🙏

[mrsjsmith]

I am full of admiration for you both and how you are coping with this sudden and terrifying experience with something I, and no doubt others here, have never heard of. I was interested to see that your husbands meds came from the UK.

Your husband is lucky to have you fighting in his corner, and he has you and his age on his side. Wishing you both well.

Thank you for the update

Colette x

[Bubnojay]

Caring for one so ill who is dearly loved is riding a roller coaster of emotions.

Oh how I hope that all goes well and you continue to find the strength to be there with love and support, and I hope that you too are getting support to help you cope.

The road you are on is hard, I am sure I am only one of many here that is thinking of you both.

Hugs and best wishes

Bubnojay

[Ghounds]

Hi, thanks for the update. I'm sure I'm not the only one who has been wondering how you both were after such a terrible shock.

I'm pleased to hear your husband's treatment so far has been successful in reducing his white cell count and spleen although the drama and reactions must have been terrifying; however the medical team sound wonderful.

Very best wishes for his ongoing treatment and recovery, hopefully you will both have some time to get your breath back.

Hang in there!

[lankisterguy Volunteer]

Hi Whatadayitwas,

-

We are sorry that you and Mike have had this tortorous journey, but relieved that you have found the expert doctors to guide you through. We hope that the worst is over for both of you and that the beast has been tamed or banished for a long time.

-

Len

[Healing2023]

Dear Whatadayitwas,

I realise this reply is two years after you post, but I have just come across this community while researching my husbands new now diagnosis of TPLL.

My husband was only re diagnosed in March2023 after being diagnosed with a different type of LPDin 2021 and the chemo they gave him for that was not effective for TPLL. He won’t though a terrible time for many months of having the wrong treatment.

It has certainly been a roller coaster for him also and myself so I felt deeply for what you went though. My husband will need a stemcell transplant also. Being this post is two years on, I sincerely hope you have had some positive outcomes.

,

[SofiaDeo in reply to Healing2023]

Sorry to hear about the misdiagnosis, then diagnosis. IDK where you are, but here is a list of some current trials re:his disease, you have to verify it's T-PLL and not B-PLL in the studies, I could not search T-PLL. There may be more trials out there, these are just ones that are reported to the people maintaining this database. If any of these researchers are near you, you might be interested in a second opinion, given the original misdiagnosis.

clinicaltrials.gov/ct2/resu...

[Healing2023 in reply to SofiaDeo]

Hi SofiaDeo,

thanks so much for replying. We are in Australia. Yes, I do worry as his wbc went way up after being indolent for a year last July,and they said CHOEP21 was right treatment… then when only PR, told us to go away and enjoy Christmas….then tried Pralatrexate, then did more tests after 7 months and changed diagnosis from PTCL to TPLL. So currently on Alemtuzumab. It has had a good effect within 4 weeks, but now other conditions are occurring, which will hold up stem cell plans until under control. I will keep researching as my husband is not up to ask for 2nd opinion at the moment.

His current haematologist has said he is too old for trials. He is in his early 60s. I will have a look at the link you put in thanks so much.

[SofiaDeo in reply to Healing2023]

Hmmm depending on the trial, I don't think early 60's is too old. Other organ systems being OK, it's not too old in the US, I was in a trial a few years ago at age 60. However, he does have a rare cancer, not a huge amount of "how to treat" data out there, and the doc may not want to spend a lot of time on unknowns. If there were even studies in AUS right now, which according to that database link, there aren't.

I took a 16 week course of alemtuzumab *subcutaneous* for my CLL back in 2012. Since it wasn't IV, I didn't have the infusion reactions. I did have an episode of rigors/shaking/chills. However, there was a small study in PLL patients and initial data showed that SC does not work well in PLL, it has to be IV.

My understanding is that this is on the "more immune suppressing" side of treatment, and infection can be a concern compared to other drugs. I had to take prophylactic antibiotic, antifungal, and antiviral therapy for mmm a year? during & after finishing the 16 weeks. I also needed a red blood cell infusion, but I was also at very high altitude (7000 feet) so riskier than at sea level, when my hemoglobin dropped. So if he's not taking those 3 anti infective prophylaxis, he probably should be. Unless there is a reason he can't, it has been considered standard protocol because this drug is closer to the "severe immune suppression" like chemotherapy is. So protection from opportunistic as well as pathogens has been recommended.

I am not sure the administrators will "ban" you, but I also don't think we can offer much help re: the drugs he may be taking. Or what his symptoms may mean, within his disease context. I am one of the few here who took alemtuzumab I think. We *can* offer tips on dealing with "life as an immune compromised person." So, discussion around infections and should one maybe see a doc urgently, how to deal with nausea and fatigue, how to avoid or talk about disease with others, diets to be healthy that don't upset our stomach, we have some insight on. Tips for easier blood draws and infusions and bone marrow biopsies, and whether or not certain labwork is normal. I personally know a bit more about T-cell diseases since I worked in places that had a number of HIV patients (a mostly T cell disease).