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T-ALL/LBL 伴 t(8;14)(q24;q11)/TCRA/D:MYC 易位患者的特征与治疗策略

英文原题:Characteristics and therapeutic approaches for patients diagnosed with T-ALL/LBL exhibiting t(8;14)(q24;q11)/TCRA/D:MYC translocation.

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Characteristics and therapeutic approaches for patients diagnosed with T-ALL/LBL exhibiting t(8;14)(q24;q11)/TCRA/D:MYC translocation.

PubMed 2023/09/07(内容时间) Leuk Lymphoma Q3 · IF 2.1(JCR 2025)

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中文摘要

伴有 t(8;14)(q24;q11)/TCRA/D::MYC 易位的 T 急性淋巴细胞白血病/淋巴瘤(T-ALL/LBL)患者构成一个罕见亚组,疾病进程具有侵袭性。在对 14 例患者进行的回顾性分析中,所有病例均在难治或复发阶段确诊(5 例为原发肿瘤,9 例为复发)。

值得注意的是,多数患者检测到髓外侵犯。4 例存在 STIL::TAL1 易位,6 例存在 CDKN2A/B 基因缺失。仅接受化疗或异基因造血干细胞移植(HSCT)的 7 例患者治疗结局均很差,最终全部死于疾病,中位总生存期(OS)仅 3 个月。6 例接受 CD7 CAR-T 治疗的患者中,5 例达到完全缓解(CR)。CAR-T 后接受 HSCT 的 4 例患者至今均无病生存期。伴 t(8;14) 易位的 T-ALL/LBL 患者预后仍不佳,但 CD7 CAR-T 治疗可能为达到 CR 提供一种潜在途径。CAR-T 后进行 HSCT 可能是实现长期生存的可行策略。

展开英文摘要原文

T-acute lymphoblastic leukemia/lymphoma (T-ALL/LBL) patients with t(8;14)(q24;q11)/TCRA/D::MYC translocation represent a rare subgroup, with an aggressive course. In our retrospective analysis of 14 patients, all were identified during refractory or relapsed stages (5 primary tumor, 9 relapse).

Notably, extramedullary invasion was detected in most patients. Four exhibited STIL::TAL1 translocation, and six demonstrated CDKN2A/B gene loss. The therapeutic outcomes were notably poor for all seven patients who received only chemotherapy or allogeneic hematopoietic stem cell transplantation (HSCT); all eventually succumbed to the disease with a median OS of 3 months.

In the application of CD7 CAR-T therapy in six patients, five achieved CR. Of the four patients who underwent HSCT following CAR-T therapy, all have remained disease-free. The prognosis for T-ALL/LBL patients with t(8;14) translocation remains bleak, but interventions involving CD7 CAR-T may offer a potential pathway to CR. HSCT following CAR-T could be a viable strategy for long-term survival.

论文信息

作者
Zhang X、Wang T、Zhang Y、Wang F、Chen J、Ni J、Sun R、Wei Z
单位
Hebei Yanda Lu Daopei Hospital, Langfang, P.R. China.China
期刊
Leukemia & lymphoma2023 Dec
原文标识
PubMed 37674391 · DOI 10.1080/10428194.2023.2254428