Journal
FRONTIERS IN ONCOLOGY
Volume 12, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fonc.2022.1013046
Keywords
acute promyelocytic leukemia; variant; BCOR-RARA; all-trans retinoic acid; allogeneic hematopoietic stem cell transplantation
Categories
Funding
- National Natural Science Foundation of China
- Science and Technology Commission of Shanghai Municipality
- [81800141]
- [81890994]
- [81770144]
- [81870110]
- [19DZ1910702]
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This study reported a rare case of BCOR-RARA variant APL and suggested that allo-HSCT after ATRA combined with chemotherapy is the optimal choice for vAPL patients with a high risk of relapse.
Acute promyelocytic leukemia (APL) is characterized by the balanced translocation of chromosomes 15 and 17, resulting in the formation of PML-RARA fusion gene. More than 98% of APL have PML-RARA fusion, and less than 2% have other types of RARA gene partners, which named variant APL (vAPL). In the present study, we reported a vAPL with BCOR-RARA, which was the third case of BCOR-RARA APL published. The patient achieved complete remission (CR) with all-trans retinoic acid (ATRA) monotherapy, and molecular CR with ATRA plus standard chemotherapy. After that, he underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) and ATRA maintenance and maintained a molecular CR status. This case provided valuable insights into the accurate identification of vAPL. Moreover, ATRA combined with chemotherapy followed by allo-HSCT was suggested as an optimal choice for those vAPL patients who had a high risk of relapse.
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