09 September 2019

A new consensus paper sets out guidelines for the diagnosis, staging, and assessment of treatment response for T-cell prolymphocytic leukaemia (T-PLL).

The clinical course of this rare aggressive type of leukaemia varies considerably between patients. Until now, the management of T-PLL has been based upon guidelines for chronic lymphocytic leukaemia, despite the differences between the two diseases. The new guidelines for T-PLL set out a consensus on how research into the disease should be conducted, so that tailored treatment regimens can be developed.

Dr Philipp Staber of Medical University of Vienna, Austria, and colleagues say: “With the advent of novel treatment options that will potentially change the management of T-PLL patients it has become necessary to produce consensus guidelines for the design and conduct of clinical trials.

 “These criteria will facilitate comparison of results from clinical trials in T-PLL and will thus support clinical decision making as well as the approval of new therapeutics by health care authorities.”

The guidelines, which appeared in the journal Blood, discusses the use of the Bcl-2 inhibitor venetoclax as a novel form of treatment. It also covers how T-PLL should be diagnosed and how treatment response can be measured.

Dr Staber says: “This work represents a milestone in the study of T-cell prolymphocytic leukaemia. This is the scientific birth of T-cell prolymphocytic leukaemia, as it were, so that we can finally find effective treatments for our patients.”

Following on from this consensus paper, a clinical study has recently been launched. It is recruiting patients from 20 countries to examining the efficacy of the drug venetoclax.

Source: Staber, P.B., Herling, M., Bellido, M., Jacobsen, E.D., Davids, M.S., Kadia, T.M., Shustov, A., Tournilhac O., Bachy, E., Zaja, F., Porkka, K., Hoermann, G., Simonitsch, I., Haferlach, C., Kubicek, S., Mayerhoefer, M., Hopfinger, G., Jaeger, U., Dearden, C. (2019) “Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia (T-PLL)”, Blood,  available from doi: 10.1182/blood.2019000402


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