How I treat Philadelphia chromosome positive acute lymphoblastic leukaemia

Abstract

The Philadelphia (Ph) chromosome is present in approximately 20-30% of adults with acute lymphoblastic leukaemia (ALL). The poor prognosis of this relatively uncommon acute leukemia has led to the rapid adoption of treatment strategies such as unrelated donor haematopoietic stem cell transplant and tyrosine kinase inhibitors into clinical practice, despite a relative paucity of randomised clinical trials. Recently, there has been a surge of interest in the underlying biology of ALL. In combination with an accumulation of more mature clinical study data in Ph+ ALL, it is increasingly possible to make more rational and informed treatment choices for patients of all ages. In this article, I review available data and indicate how I personally interpret current evidence to make pragmatic treatment choices with my patients, outside of clinical trials. My strongest recommendation is that all physicians treating this rare disease actively seek appropriate clinical trials for their patients, wherever possible.