Sudden onset of the blastic phase of XXXX : patterns and implications.

@entity1 @entity8 @entity1148 @entity5 @entity2213 @entity1860 @entity2459
BACKGROUND: Therapeutic options in @entity1860 ( @entity1860 ) in chronic phase include allogeneic @entity8 ( @entity8 ) and treatments other than allogeneic @entity8 . The choice of therapy often depends on the mortality risk of allogeneic @entity8 . Delaying @entity8 until the disease demonstrates resistance to other strategies (e.g., @entity2213 or interferon-alpha) entails the risk of the development of a sudden blastic phase, which may not permit enough time to perform a @entity8 . METHODS: The current study was performed to define the incidence and patterns of sudden-onset blastic-phase @entity1860 in relation to the risk inherent in delaying allogeneic @entity8 . Of a study group of 1286 @entity1 who were referred in early chronic-phase @entity1860 between 1981-2001 and who received therapies other than allogeneic @entity8 , 1093 @entity1 had regular follow-up at the study institution. A sudden onset of blastic phase was defined as having its onset within 3 months from a previously documented complete hematologic response. RESULTS: Among the 1093 @entity1 , 183 @entity1 developed blastic-phase disease, which was of sudden onset in 46 @entity1 (25%; 4% of the total). The incidence of sudden blastic phase was 0.4% in the first year, 1.8% in the second year, and 2.6% in the third year. @entity1 with @entity2459 were found to have more often low-risk features at the time of presentation, including low incidences of blasts and @entity1148 . @entity2459 was found to be associated with a higher incidence of lymphoid blastic morphology (67% vs. 22%; P < 0.0001), which resulted in a higher response rate to blastic-phase therapy (70% vs. 29%; P < 0.0001) and significantly longer survival from the onset of blastic-phase disease (median of 12 months vs. 6 months; P < 0.01). Twenty-four of the 42 @entity1 with @entity2459 who were treated at the M. D. Anderson @entity5 Center were able to undergo allogeneic @entity8 in second chronic phrase (n = 13) or transformed phase (n = 11); at the time of last follow-up, 6 @entity1 were alive without evidence of disease at a median of 18 months. CONCLUSIONS: The low rates of @entity2459 in the first 3 years of the course of disease for @entity1860 and the salvage rate of these @entity1 with allogeneic @entity8 should be considered in relation to the transplantation mortality in @entity1 with early chronic-phase disease and the early promising results with @entity2213 therapy.
