Division of Pediatric Hematology-Oncology, St. Joseph's Children's Hospital, Paterson, NJ.
Division of Pediatric Hematology-Oncology, University of California, San Diego/Rady Children's Hospital San Diego, San Diego, CA.
J Pediatr Hematol Oncol. 2021 Mar 1;43(2):e246-e249. doi: 10.1097/MPH.0000000000001772.
A 6-year-old girl with a history of heart transplantation was diagnosed with myelodysplastic syndrome, which progressed to acute myelogenous leukemia. Comprehensive genomic profiling of her tumor discovered an MLL-PTD (partial tandem duplication) and she received chemotherapy and a hematopoietic stem cell transplant (HSCT). She subsequently relapsed and tumor molecular profiling was repeated, revealing 2 new potentially targetable mutations (FLT3 and IDH2). A novel treatment regimen targeting these mutations with sorafenib and azacitidine without using cytotoxic chemotherapy produced remission and she subsequently pursued a second HSCT. She remains disease-free 17 months after HSCT. This case report demonstrates how repeated tumor molecular profiling provided novel actionable information for the diagnosis and management at 2 timepoints.
一位 6 岁女孩,有心脏移植病史,被诊断为骨髓增生异常综合征,随后进展为急性髓系白血病。对其肿瘤进行全面基因组分析发现存在 MLL-PTD(部分串联重复),她接受了化疗和造血干细胞移植(HSCT)。随后她复发,再次进行肿瘤分子谱分析,发现 2 个新的潜在可靶向突变(FLT3 和 IDH2)。采用索拉非尼和阿扎胞苷靶向这些突变而不使用细胞毒性化疗的新治疗方案产生了缓解,随后她进行了第二次 HSCT。在 HSCT 后 17 个月,她仍然无疾病状态。本病例报告表明,肿瘤分子谱分析在 2 个时间点如何为诊断和治疗提供新的可操作信息。