T-PLL

Cell Markers

Cell Markers

T-PLL or t-cell prolymphocytic leukemia is a post-thymic form of chronic lymphocytic leukemia usually characterized as “aggressive, invariably fatal, with a median survival of 7.5 months”.  It affects men more than women and is more common in people in their 50′s and 60′s.

Before the introduction of Campath (alemtuzumab) in May 2001 response rates to conventional therapies was 30% at best.  Campath is classified as a biotherapy (as opposed to chemotherapy).  It is a specifically known as a monoclonal antibody. The defective lymphocytes express plentiful protein binding sites on the cell membrane surface.  The particular sites of interest are called CD52.  Campath locks onto these sites and destroys the cell.

Campath was developed as a second line treatment for the more common B-cell form of CLL.  There is a potential market of 16,000 new cases of b-cell CLL per year.  The number of new cases of t-pll is only 125-150.  Ironically t-cell disease is the more responsive to Campath.  Use of Campath for t-pll remains “off label”.

Some studies are seeking to show an improved survival profile by pairing Campath with the purine-analogue chemotherapy, pentostatin.  Reoccurrence of the disease, even after sucessful remission with Campath, is likely.  Some promise has been shown in following a successful remission with a stem cell (or bone marrow) transplant.  Transplant is theoretically a potential cure.

The journal of my struggle with this disease is offered as a free e-book at Diary of an Illness – A Cancer Nurse Battles a Rare Leukemia

* Please feel free to contact the author about t-pll questions:  Dennis (beingcancer@att.net)

** More comprehensive information on t-pll and on Campath is available at T-PLL Support Net.

T-cell prolymphocytic leukemia – Wikipedia, the free encyclopedia

Share

Comments

T-PLL — 5 Comments

  1. Hi Dennis,

    Hope all is well in your corner of the world and wish you and yours a Happy and Healthy 2010.

    Wanted to touch base with you on your experience with receiving a DLI post transplant.

    My husband is at Day 140 post MUD Allo transplant – marrow not peripheral blood – done at Roswell Park Cancer Institute in Buffalo, NY. After his last bone marrow biopsy the Doctor informed us that the myeloid compartment of the chimerism is moving in the wrong direction – was at 12% and dropped to 6%. First the NP said Max would receive DLI over the next four months. Then the doc comes in and says he spoke to our Leukemia expert (Dr. Myron Czuczman) and they decided to do nothing but wait, repeat the BMB and see if the small population (1%) of bad lymphocytes will go away. His counts have been very slow to come back up and only got to normal wbc in the past few weeks. He was taken off all immune suppression (tacro) in mid November and seemed to be doing well. He has a slight rash but no overt signs of GVHD according to the doctors and nurses that looked at the rash.

    So my question is what can you tell me about the DLI experience? Did you have a lack of engraftment of the lymphoid compartment when you received your DLI? Sounded like it is done outpatient, is that how yours was done? The myeloid is 100% donor. Is that a good sign? can’t keep wondering if we are in for more bad news at this time – meaning – seeing T-PLL relapse. Doc also mentioned worst case scenario of a second transplant – don’t know if we can go through this again as it was grueling as you know.
    Any insights would be greatly appreciated as I struggle to understand all this medical jargon.

    Sincerely,

    Shirley Fischer
    Buffalo, NY

  2. T-cell PLL is a rare leukemia and finding new treatments for patients with PLL is difficult. If you are a patient with T-cell PLL, new trials for this disease are posted on clinicaltrials.gov This website is a great resource to find new treatment options which hope to improve curing this rare disease.

  3. My 44 year old sister was admitted to the hospital on December 10, 2011 because of a cough that she could not get rid of that had turned to double pneumonia. On December 19, 2011, we were told that what she had was a very rare type of leukemia called T cell PLL. We were shocked beyond measure. After immediately turning to the WWW to read about this crazy diagnosis, we were all in awe!! How could something that began as a cough end up being a death sentence. My sister decided she would fight this monster with everything in her and fight she did. She spent about a month in Little Rock at the cancer research hospital receiving Campath treatments then was sent home to Rogers, Arkansas. She had a few good days and fought the fight with everything in her. After receiving some kind of treatment or procedure almost daily, outpatient and inpatient, she was sent to Barnes Jewish in St. Louis. Campath treatments were not longer working, so a new chemo was introduced called CHOP. On April 19th, one day after my sister’s 45th birthday and four months after the diagnosis, she transitioned into eternity. She said she would never give up, but when the cancer went to her spinal fluid, thus affecting her brain, she had nothing left to fight with and her time on earth came to an end. I hate this disease. It has been almost six weeks and I miss my sister like crazy.

  4. In Jan 2011 after a routine annual physical my wife was called and told her WC was slightly above the normal it was 16 the normal is 4 – 11. After seeing a haematologist it was initially diagnosed as CLL but further testing during the next few months gave us the bad news that she had Tpll. As she had no symptoms it was decided not to start treatment until that time. By early Dec. she was getting a lot of discomfort in her spleen and blood tests showed the WC at 330, treatment with Campath commenced on Dec 23rd it did not go well the WC continued to increase although more slowly. During the treatment time she felt very ill and weak and we had several visits to the ER to try and stableise her condition. During one of these visits in March her WC had reached 900 and the Dr`s. thought she would not live much longer so she was given Hydroxiria as a desperate measure to knock down all her counts. It worked dramatically and by the end of April she went from not being able to walk much more the 6ft to hiking 6 or more miles at a time.
    The remission lasted from April to the end of July 2012 while on a trip she again noticed pain in her spleen and tests showed not only had the WC increased to 30 from 3 but Lymphomas had grown around the blood vessels in the stomach. She again started Hydroxiria and later a 5 day course of Cladribine in the hope of reducing the Lymphomas and slowing the WC rise, but it was not to be and after all the suffering she passed away on Oct 3rd 2012 probably of an infection caused by the compromised immune system.

  5. same diagnosis as mother, routine blood work showed elevated wbc, monitored from 25k to 40-50-now 94k, very progressive but no symptoms, told prognosis 6-12 mos max after 100k wbc

Leave a Reply