BCR-ABL1 Transcript and Philadelphia Negative T-Cell Lymphoblastic Lymphoma: A Case Report and Literature Review

Research Square (Research Square)(2023)

引用 0|浏览2
暂无评分
摘要
Abstract Background: Lymphoblastic lymphoma (LBL) is a malignant tumor, with most cases derived from T cells and less than 20% arising from B cells. T-LBL originates from immature T cell precursors or lymphoblasts. It is mainly found in the lymph nodes, and the bone marrow is involved in less than 25%. The Philadelphia chromosome (Ph) is the first cytogenetic abnormality related to chronic myeloid leukemia (CML), a human tumor. Together with CML, a derivative rearrangement of the chromosomes between 9 and 22, containing the BCR-ABL1 fusion gene, can be detected in acute lymphoblastic leukemia (ALL). However, this is rarely reported in T-cell ALL/LBL cases. Herein, we report a case of T-LBL with a normal chromosomal karyotype but with a BCR-ABL1 fusion transcript. Case presentation: A 72-year-old male with no remarkable medical history was admitted with cervical lymph node enlargement of more than 1 month. Physical examination revealed several enlarged bilateral lymph nodes in the neck and supraclavicular fossae. The patient underwent neck lymphadenectomy, with two lymph nodes completely resected. Pathological assessment and flow cytometry immunophenotypic analysis were suggestive of infiltration by lymphoma cells. Two weeks following induction therapy (mini-hyper-CVD [C:CTX, cyclophosphamide, V:VCR, vincristine, D:DXM, dexamethasone] combined with venetoclax), flow cytometry immunophenotypic analysis of bone marrow cells revealed measurable residual disease, and real-time polymerase chain reaction (RT-PCR) revealed that BCR-ABL1 rearrangement decreased from 35.13% to 2.71%. The second regimen consisted of methotrexate + cytarabine combined with tyrosine kinase inhibitors. The patient then received targeted drugs combined with chemotherapeutic drugs as maintenance therapy and was in complete remission for 16 months following diagnosis. Positron emission tomography revealed a complete metabolic response after therapy for 4 and 15 months, and RT-PCR revealed that BCR-ABL1 rearrangement was 0.04% after 15 months of therapy. Conclusions: For older adults with lymphoblastic leukemia/lymphoma, mini-hyper-CVD combined with other novel drugs provides a promising treatment approach; however, the subtypes of the disease, patient age, and comorbidity profile are all important variables to consider. Further, fluorescence in situ hybridization and RT-PCR testing performed on bone marrow and lymph node specimens for BCR-ABL1 rearrangements as part of routine diagnostic tests are recommended.
更多
查看译文
关键词
lymphoma,bcr-abl,t-cell
AI 理解论文
溯源树
样例
生成溯源树,研究论文发展脉络
Chat Paper
正在生成论文摘要