Meduni Vienna: Success in the treatment of aggressive lymphatic cancer

(Vienna, 15th October 2012) ALCL (anaplastic large cell lymphoma) is a particularly aggressive form of lymphatic cancer. This type of cancer most commonly occurs in children and young adults and so far has been difficult to treat. Now, a group led by Lukas Kenner from the MedUni Vienna and the Ludwig Boltzmann Institute, with assistance from Karoline Kollmann and Veronika Sexl from the Vienna University of Veterinary Medicine and an international group of researchers, has successfully treated a seriously ill ALCL patient with a specific PDGFR inhibitor. Says Kenner: “In just ten days, the 27-year-old patient was free from tumours and has been leading a completely normal life for more than 22 months. Without this treatment, the patient would most probably be already dead.”

It was already known that patients with this form of lymphatic cancer possess a certain genetic defect which activates the NPM-ALK gene (nucleophosmin-anaplastic lymphoma kinase). NPM-ALK has long since been associated with the development of cancer. What was not known until now, however, was just what role this gene played in the development of the condition.

Daniela Laimer and Helmut Dolznig from the MedUni Vienna, along with Paul Vesely from the MedUni Graz, also played a very important role in the study, which has now been published in the highly respected journal “Medicine Nature”. The scientists discovered that this gene triggers the production of two transcription factors that activate a specific protein.

The researchers were able to inhibit this protein (PDGFRB / platelet-derived growth factor receptor B) using the substance Imatinib, which is already known about. This significantly increases the patient’s life expectancy – as has been demonstrated through in vivo trials.

At the present time, ALCL lymphatic cancer is usually treated with chemotherapy and bone marrow transplant, but in view of the aggressiveness of the disease, many patients suffer a life-threatening relapse after an initially successful response to treatment.

“Our results invite the conclusion that patients who are no longer responding to chemotherapy will benefit from treatment with Imatinib,” says Kenner. “Our patient had run out of treatment options and would most likely have died a long time ago,” says Kenner. “This form of treatment could generally markedly improve survival rates.” Other patients suffering from aggressive lymphoma with active PDGFRB have also since been successfully treated as part of a cooperation with the University Hospital in Bologna.

Service: Medicine Nature
“Identification of PDGFR blockade as a rational and highly effective therapy for NPM-ALK driven lymphomas”; Daniela Laimer, Helmut Dolznig, Karoline Kollmann, Paul W. Vesely, Michaela Schlederer, Olaf Merkel, Ana-Iris Schiefer, Melanie R. Hassler, Susi Heider, Lena Amenitsch, Christiane Thallinger, Philipp B. Staber, Ingrid Simonitsch-Klupp, Matthias Artaker, Sabine Lagger, Stefano Pileri, Pier Paolo Piccaluga, Peter Valent, Katia Messana, Indira Landra, Thomas Weichhart, Sylvia Knapp, Medhat Shehata, Maria Todaro, Veronika Sexl, Gerald Höfler, Roberto Piva, Enzo Medico, Bruce A. Riggeri, Mangeng Cheng, Robert Eferl, Gerda Egger, Josef M. Penninger, Ulrich Jaeger, Richard Moriggl, Giorgio Inghirami and Lukas Kenner. 

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