儿童肝脾T细胞淋巴瘤2例并文献复习
收稿日期: 2024-10-08
录用日期: 2025-03-13
网络出版日期: 2025-08-27
基金资助
湖北省自然科学基金-青年项目(2019CFB368)
Two cases of pediatric hepatosplenic T-cell lymphoma and literature review
Received date: 2024-10-08
Accepted date: 2025-03-13
Online published: 2025-08-27
目的 探讨肝脾γδT细胞淋巴瘤（HSγδTCL）患儿的临床特征、治疗及预后。方法 回顾性收集2012年10月至2023年5月我院肿瘤科收治的2例HSγδTCL患儿的临床资料，并检索相关文献进行文献复习。结果 2例患儿分别为9、11岁男童，以脾大及血细胞减少起病，病理类型均为HSγδTCL。例1在诊断后给予化学治疗，因病情进展死亡。例2经过7个疗程的化学治疗及联合组蛋白去乙酰化酶抑制剂治疗，缓解后行异基因造血干细胞移植，目前存活。共检索到中英文报道的30例患儿，其中8例既往有慢性免疫性疾病病史，并接受过免疫抑制剂治疗；11例接受高强度的化疗联合造血干细胞移植，5例接受脾脏切除术及化疗；22例患儿死亡，诊断后中位生存时间短于1年。结论 本病治疗效果差，化疗联合造血干细胞移植是可选择的治疗方法。
关键词： 肝脾γδT细胞淋巴瘤; 儿童; 化疗; 造血干细胞移植
汪洁 , 吴彬 , 张兰男 , 陈开澜 . 儿童肝脾T细胞淋巴瘤2例并文献复习[J]. 临床儿科杂志, 2025 , 43(9) : 698 -704 . DOI: 10.12372/jcp.2025.24e1059
Objective To investigate the clinical characteristics, treatment outcomes, and prognosis of hepatosplenic γδ T-cell lymphoma (HSγδTCL) in pediatric patients. Methods The clinical data of two pediatric patients with HS γδ TCL admitted to the oncology department of our hospital from October 2012 to May 2023 were retrospectively analyzed. Additionally, relevant literature was systematically searched and reviewed. Results The two patients were 9- and 11-year-old boys who presented with splenomegaly and cytopenia. Both were diagnosed with HSγδTCL. Patient 1 received chemotherapy but succumbed to disease progression. Patient 2 underwent seven cycles of chemotherapy in combination with histone deacetylase inhibitor therapy. After achieving remission, the patient received allogeneic hematopoietic stem cell transplantation and remains alive to date. A total of 30 pediatric cases were reviewed from the literature. Eight patients had a history of chronic immunodeficiency diseases and had received immunosuppressive therapy. Eleven patients underwent intensive chemotherapy combined with stem cell transplantation, while five patients had splenectomy followed by chemotherapy. Overall, 22 patients died, with a median survival time of less than one year after diagnosis. Conclusion HSγδTCL in children has a poor treatment outcome. Chemotherapy combined with hematopoietic stem cell transplantation is an alternative treatment strategy worthy of consideration.
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