Treatment of Philadelphia-positive acute lymphoid leukemia

SUMMARY Philadelphia-positive (Ph+) acute lymphoblastic leukemia (ALL) represents approximately 15–30% of ALL in adults and is characterized by the expression of the fusion protein BCR–ABL with oncogenic activity. Remission and survival rates were lower whereas relapse risk was increased in Ph+ compared with Philadelphia-negative ALL, until remarkable improvements in the management of Ph+ ALL were achieved through the introduction of tyrosine kinase inhibitors that reduce the activity of the BCR–ABL protein. However, in patients achieving complete remission, allogeneic hematopoietic stem cell transplantation is in most cases a mandatory therapeutic step because rate of relapses are still high. This review will illustrate the current therapeutic options for the management of Ph+ ALL and indicate how better curative options may stem from appropriate implementation of tyrosine kinase inhibitors and novel antitumoral agents.