Management of CML-blast crisis

- BCR-ABL is the driving force of progression.
- Clonal evolution indicates progression.
- BC patients who achieve a 2nd CP have the best prognosis.
- Allo-SCT should be tried in 2nd CP whenever a donor is available and the patient can tolerate the procedure.
- BC can be prevented by elimination (or reduction) of BCR-ABL.
- Much is known on genetic instability and clonal evolution as causes of BC, but confirmation of our understanding by successful intervention as proof of principle is lacking.

Tyrosine kinase inhibitors (TKI) have moderately improved survival in BC, but a median survival of less than 1 year is still unsatisfactory. This article reviews the various tests required for diagnosis of BC, features at diagnosis, treatment modalities (intensive chemotherapy, TKI, allo-SCT and a selection of investigational agents), options of prevention and predictors of progression. The best prognosis is observed in patients that achieve a 2nd CP. Allo-SCT probably further improves prognosis of patients in 2nd CP. The choice of TKI should be directed by the mutation profile of the patient. BC can be prevented. A careful analysis of risk factors for progression may help. Current treatment options are combined in a concluding strategy for the management of BC.