T-PLL
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
cancer
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
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.