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 (firstname.lastname@example.org)
** More comprehensive information on t-pll and on Campath is available at T-PLL Support Net.