Journal
FRONTIERS IN ONCOLOGY
Volume 10, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fonc.2020.611467
Keywords
fusion gene; STRBP-JAK2; Ph-like; chimeric antigen receptor; B-lymphoblastic leukemia
Categories
Funding
- National Natural Science Foundation of China [81873443, 81700139]
- National Science and Technology Major Project [2017ZX09304021]
- National Key R&D Program of China [2016YFC0902800]
- Priority Academic Program Development of Jiangsu Higher Education Institutions (PAPD)
- Frontier Clinical Technical Project of the Science and Technology Department of Jiangsu Province [BE2017655]
- Jiangsu Provincial Medical Talent [ZDRCA2016045]
- Major Natural Science Research Projects in institutions of higher education of Jiangsu Province [19KJA210002]
- Key Science Research Project of Jiangsu Commission of Health [K2019022]
- National Science Fund of Jiangsu Province [BK20170360]
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This study reported a novel STRBP-JAK2 gene fusion in a Ph-like ALL patient with an aggressive disease course, resistant to chemotherapy combined with ruxolitinib but sensitive to immunotherapy. It suggests that CAR T-cell therapy may be a viable option for this type of leukemia.
Philadelphia chromosome-like B-lymphoblastic leukemia (Ph-like ALL) describes a group of genetically heterogeneous, Ph-negative entities with high relapse rates and poor prognoses. A Janus-kinase-2 (JAK2) rearrangement has been reported in approximately 7% of Ph-like ALL patients whose therapeutic responses to JAK inhibitors have been studied in clinical trials. Here, we report a novel STRBP-JAK2 fusion gene in a 21-year-old woman with Ph-like ALL. Although a normal karyotype was observed, a hitherto unreported JAK2 rearrangement was detected cytogenetically. STRBP-JAK2 fusion was identified by RNA sequencing and validated by Sanger sequencing. The Ph-like ALL proved refractory to traditional induction chemotherapy combined with ruxolitinib. The patient consented to infusion of autologous chimeric antigen receptor (CAR) T cells against both CD19 and CD22, which induced morphologic remission. Haplo-identical stem cell transplantation was then performed; however, she suffered relapse at just one month after transplantation. The patient subsequently received donor lymphocyte infusion after which she achieved and maintained a minimal residual disease negative remission. However, she succumbed to grade IV graft-versus-host disease 7 months post-transplant. In conclusion, this report describes a novel STRBP-JAK2 gene fusion in a Ph-like ALL patient with a very aggressive disease course, which proved resistant to chemotherapy combined with ruxolitinib but sensitive to immunotherapy. Our study suggests that CAR T-cell therapy may be a viable option for this type of leukemia.
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