【摘要】 目的 探讨自体造血干细胞移植(autologous hematopoietic stem cell transplantation,auto-HSCT)治疗侵袭性NK/T细胞淋巴瘤的疗效。 方法 对我科2005年1月16日收治的1例侵袭性NK/T细胞淋巴瘤患者的造血干细胞移植和随访资料进行回顾性分析,并复习国内外相关文献。 结果 患者为37岁女性,诊断结外鼻型NK/T细胞淋巴瘤,系统性,经CHOAP和ICE方案化学疗法、手术、局部放射治疗控制病情良好后,采集自体骨髓造血干细胞,行auto-HSCT,预处理方案为全身放射治疗+ECy;移植+29 d造血功能即顺利重建;移植后密切随访,患者一直处于完全缓解,至今已存活67个月。 结论 auto-HSCT治疗侵袭性NK/T细胞淋巴瘤疗效肯定、可靠。【Abstract】 Objective To explore the therapeutic effect of autologous hematopoietic stem cell transplantation (auto-HSCT) on aggressive NK/T lymphoma. Methods The clinical data of one patient with aggressive NK/T lymphoma diagnosed in January 2005 were retrospectively analyzed, and the relevant domestic literatures were analyzed. Results This thirty-seven-year-old female patient had good disease control after undergoing chemotherapy with CHOAP and ICE regimens, surgery, and locoregional radiotherapy. After that, she had been collected enough bone marrow-derived hematopoietic stem cells, then underwent auto-HSCT with these cells. The conditioning regimen was TBI plus ECy. On the +29th day after transplantation,the hematopoietic reconstruction was successful. During the follow-up period, the patient was in complete remission status all along and her disease-free survival (DFS) was 67 months. Conclusion Auto-HSCT is effective on aggressive NK/T lymphoma.
目的:探讨肝脾γδT细胞淋巴瘤的临床表现、病理学特征、免疫表型特点。方法:对我院2例确诊的肝脾γδT细胞淋巴瘤患者的临床资料进行分析、追踪随访并进行文献复习。 结果:该组患者均为青年男性,肝脾不同程度长大,发热,全血细胞减少(1/2),肝功能受损,淋巴结未受累;病理示瘤细胞弥漫性肝、骨髓的窦内侵犯;免疫表型:患者瘤细胞表达CD2(+)、CD3(+)、CD56(+)、CD16(+)、CD20()、TIA1(+)、TCRγ/δ(+)。结论:肝脾γδT细胞淋巴瘤是较为罕见的外周T细胞淋巴瘤,以肝脾长大、发热为主要临床表现,通常淋巴结不受累,病情进展快,疗效差,生存期短。
In recent years, the complexity of intraocular lymphoma has been gradually recognized by ophthalmologists. Although primary vitreoretinal lymphoma is the dominant type of intraocular lymphoma, ophthalmologists should be aware that it is not unique and avoid overgeneralizing specific clinical features to all intraocular lymphoma types. Intraocular lymphoma can be divided into vitreoretinal, uveal (choroid, iris, ciliary body) lymphoma according to the anatomic affected parts. According to pathological cell types, it can be divided into B cells, mantle cells, T cells and natural killer T cells. At the same time, depending on the presence or absence of extra-ocular tissue involvement, it can also be subdivided into isolated intraocular, oculo-central nervous system, oculo-system, and oculo-central nervous system lymphomas. Vitreoretinal lymphoma tends to occur in the elderly with clinical manifestations similar to uveitis and white spot syndrome and limited response to glucocorticoid therapy. The characteristic fundus manifestations include vitreous gauzy or "auroral" opacity and yellowish-white subretinal mass. Optical coherence tomography plays a key role in diagnosis and can reveal specific changes such as vertical strong reflex and intraretinal strong reflex infiltration. It is worth noting that vitreous and retinal involvement may vary, which has guiding significance for the selection of treatment strategies. In contrast, uveal lymphoma has unique clinical and pathological features, such as the chronic course of choroidal mucosa-associated lymphoid tissue (MALT) lymphoma and the equal distribution of T cells and B cells in iris lymphoma. In diagnosis, choroidal lymphoma often requires histopathological examination, and radiotherapy is the first choice for MALT lymphoma. T-cell lymphoma is similar to B-cell lymphoma in ocular fundus appearance, but diagnosis is more difficult and depends on cytopathology and T-cell receptor gene rearrangement. Comprehensive systematic screening is essential for patients with intraocular lymphoma to identify the primary site. Ocular lesions in patients with systemic lymphoma require differential diagnosis, including tumor invasion, secondary infection, and inflammatory lesions. As the incidence of lymphoma increases, ophthalmologists should constantly update their understanding of intraocular lymphoma to provide accurate diagnosis and treatment.