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Identification of STRBP as a Novel JAK2 Fusion Partner Gene in a Young Adult With Philadelphia Chromosome-Like B-Lymphoblastic Leukemia
- Source :
- Frontiers in Oncology, Vol 10 (2021)
- Publication Year :
- 2021
- Publisher :
- Frontiers Media SA, 2021.
-
Abstract
- 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.
- Subjects :
- Oncology
Cancer Research
medicine.medical_specialty
Ruxolitinib
Philadelphia chromosome
lcsh:RC254-282
Donor lymphocyte infusion
Fusion gene
03 medical and health sciences
0302 clinical medicine
hemic and lymphatic diseases
Internal medicine
medicine
B-lymphoblastic leukemia
chimeric antigen receptor
business.industry
Induction chemotherapy
Ph-like
lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens
medicine.disease
STRBP-JAK2
Chimeric antigen receptor
Transplantation
Leukemia
fusion gene
030220 oncology & carcinogenesis
business
030215 immunology
medicine.drug
Subjects
Details
- ISSN :
- 2234943X
- Volume :
- 10
- Database :
- OpenAIRE
- Journal :
- Frontiers in Oncology
- Accession number :
- edsair.doi.dedup.....d78156a9a52054aa4bf9f11aa44b492b