Clinical analysis of 82 cases of acute promyelocytic leukemia with short isoform in children and adults.

BACKGROUND

Acute promyelocytic leukemia (APL) with fusion gene is a distinct variant of acute myeloid leukemia. According to the different break sites of the gene, there are three transcripts: Long (bcr1), Variant (bcr2) and Short (bcr3).

METHODS

We retrospectively analyzed 82 APL cases with short isoform.

RESULTS

A total of 384 patients with APL were seen, of which 85(22.14%) had short isoform (bcr3) and 82 met the inclusion criteria. The median age was 33.5 years (range, 2-72 years). The incidences of hemorrhage in the intermediate- and high-risk group were higher, but only the incidence between medium and low risk differed statistically (, and the incidences of fever, fatigue, splenomegaly, and lymph node enlargement and differentiation syndrome (DS in those groups were not statistically significant (>0.05). gene mutation rate and the mortality rate of the high-risk group were significantly higher than that of other groups (=0.040 and =0.004, 0.041 and =0.037, respectively). The mortality rate was lowest (4.26%) in the group treated with ATRA combined with arsenic and anthracycline. The 3-year OS and the 3-year DFS of the low and intermediate-risk group were better (=0.019 and =0.017, respectively).

CONCLUSIONS

ATRA combined with arsenic and anthracycline had significant impact on outcomes in APL with short isoform.