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EsPhALL2017

Acute lymphoblastic leukaemia, ALL for short, is the most common form of blood cancer in children and adolescents. The disease begins in the bone marrow, where the normal process of blood formation is disrupted by the uncontrolled proliferation of immature blood cells. Untreated, ALL leads to serious illness and impairment of the organs with a fatal outcome.

Philadelphia chromosome-positive acute lymphoblastic leukaemia, Ph+ALL for short, is an ALL involving a specific genetic mutation. As a result of this mutation, an enzyme is produced in the body that is responsible for the malignant properties of these special leukaemia cells and activates their excess reproduction. For this reason, Ph+ALL requires a special therapy consisting of a very intensive combination chemotherapy with medicines that have different actions. The current standard therapy has already improved the chances of survival for Ph+ALL patients considerably. The main problems with this intensive therapy, however, are the life-threatening side effects and the serious long-term complications associated with the medication. Approximately five children and adolescents are affected by this disease each year in Switzerland and are eligible to participate in the EsPhALL2017 study.

The aim of EsPhALL2017 is to further improve the treatment of patients with Ph+ALL. The aim on the one hand is to reduce the side effects of the therapy as far as possible by reducing the intensity and duration of therapy. On the other hand, however, each child should be treated as often and for as long as is necessary to overcome the leukaemia so that it does not recur. In order to account for these different aims in each patient as well as possible, treatment is given in so-called risk groups where it can be adapted to the respective risk of recurrence. A standardised diagnostic workup in reference laboratories will be performed so that the treatment can be studied correctly and in great detail, and medical data will be recorded, stored and jointly evaluated for all participating patients. In addition, research projects will be carried out to continuously improve our understanding of the biology of Ph+ALL in children and adolescents and to research new approaches to diagnosis and therapy.

The University Milano-Bicocca in Italy is responsible for the international implementation of the study as sponsor. The Swiss Paediatric Oncology Group (SPOG) is responsible for performing the study in Switzerland (sponsor representative).

In short

  1. The aim of the study is to improve the treatment of patients with a specific form of genetically mutated acute lymphoblastic leukaemia (ALL). The biology of this form of ALL is also being investigated in order to acquire a better understanding of the disease in children and adolescents and explore new approaches to diagnosis and therapy.
  2. The current standard therapy has already greatly improved the chances of survival for children and adolescents with this illness. The main problems with this intensive therapy, however, are the life-threatening side effects and the serious long-term complications associated with the medication.
  3. The aim of the study is to reduce the intensity and duration of the treatment while still guaranteeing recovery. The treatment will be modified in different risk groups based on the individual relapse risk in order to find the balance between side effects and successful treatment.
Published 03.05.2021
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