γδ T细胞淋巴瘤的免疫学和临床特征

　　γδ T细胞淋巴瘤非常罕见。在世界范围内约占侵袭性T细胞淋巴瘤的1%。γδ T细胞淋巴瘤具有独特的表型，通常CD4/CD8双阴性表达，CD56表达，T细胞受体有一条γ链，表达细胞毒性相关蛋白（如TIA-1）。

　　原发性皮肤γδ T细胞淋巴瘤与嗜血细胞综合征(HPS)的关系

　　目前还不清楚γδ T细胞淋巴瘤合并HPS是否与肿瘤分泌细胞因子有关。但是，合并HPS的T细胞淋巴瘤预后不良，甚至会导致一些患者死亡。

　　γδ T细胞淋巴瘤的鉴别诊断要点

　　γδ T细胞淋巴瘤诊断很难，在国际外周T细胞淋巴瘤的研究项目中，对于皮肤和肝脾γδ T细胞淋巴瘤的诊断，只有75%病理学家可达成一致意见。此外，T细胞淋巴瘤侵犯骨髓，使肝脾γδ T细胞淋巴瘤的病理诊断很难，只能靠特别的染色手段检出。使用抗体或通过流式细胞仪寻找γδ很重要，否则可能诊断错误。

　　γδ T细胞淋巴瘤的治疗方案

　　目前没有很好的γδ T细胞淋巴瘤治疗方案。肝脾γδ T细胞淋巴瘤多采取核苷类似物为基础的方案治疗（如喷司他丁），如果治疗有反应，下一步是异基因造血干细胞移植，这是唯一能治愈的方法。皮肤γδ T细胞淋巴瘤的侵袭性强，常接受多药化疗，如果这种治疗有反应，下一步还是异基因造血干细胞移植，这也是唯一能治愈的方法。不幸的是，目前没有大量新药治疗γδ T细胞淋巴瘤的数据。因为在临床试验这些患者被排除在外。目前来看，Stat抑制剂、抗CCR4抗体药也许会有些治疗效果。

 访谈原文

　　Oncology Frontier:  What are the immunophenotype and clinic featuresof γδT-cell lymphoma?

　　《肿瘤瞭望》：γδ T细胞淋巴瘤的免疫学和临床特征是怎样的？

　　Foss博士: The γδT-cell lymphomas are very rare. They make up about 1% of all aggressive T-cell lymphomas around the world. They have a unique phenotype in that they are CD4- and CD8-negative and they do express， of course， a γ-chain of the T-cell receptor. They often also express cytotoxic markers such as TIA-1. In addition， they can express CD56 as well.

　　Foss博士：γδ T细胞淋巴瘤非常罕见。在世界范围内约占侵袭性T细胞淋巴瘤的1%。γδ T细胞淋巴瘤具有独特的表型，通常CD4/CD8双阴性表达，CD56表达，T细胞受体有一条γ链，表达细胞毒性相关蛋白（如TIA-1）。

　　Oncology Frontier:  Primary cutaneous γδT-cell lymphoma is often complicated by the hemophagocytic syndrome (HPS). Can you explain this association?

　　《肿瘤瞭望》：原发性皮肤γδ T细胞淋巴瘤通常由于嗜血细胞综合征(HPS)变得复杂，能否请您解释二者之间的关系？

　　Dr Foss: The γδT-cell lymphomas are associated with hemophagocytosis that may or may not have something to do with cytokine secretion by the tumors， but we don’t really know. We do know that when hemophagocytosis occurs that it is a very poor prognostic feature of these tumors. In fact， that can then be the cause of death in some patients with this disease.

　　Foss博士：目前还不清楚γδ T细胞淋巴瘤合并HPS是否与肿瘤分泌细胞因子有关。但是，合并HPS的T细胞淋巴瘤预后不良，甚至会导致一些患者死亡。

　　Oncology Frontier:  What are the differential diagnoses for γδT-cell lymphoma that should be considered?

　　《肿瘤瞭望》：γδ T细胞淋巴瘤的鉴别诊断要点有哪些？

　　Dr Foss: The γδT-cell lymphomas are difficult to diagnose. If you look at the International Peripheral T-cell Lymphoma Project， there was only about a 75% concordance among pathologists for the diagnosis of γδT-cell lymphoma of the skin and hepatosplenic T-cell lymphoma (which is the other major γδT-cell lymphoma). Also they can be difficult to diagnose histopathologically because， in the case of hepatosplenic T-cell lymphoma， there is often infiltration of the sinusoids of the bone marrow. That may not be readily apparent unless you use special stains. It is important to actually look for γδ using the antibody that is available or by flow cytometry， otherwise the diagnosis may be missed.

　　Foss博士：γδ T细胞淋巴瘤诊断很难，在国际外周T细胞淋巴瘤的研究项目中，对于皮肤和肝脾γδ T细胞淋巴瘤的诊断，只有75%病理学家可达成一致意见。此外，T细胞淋巴瘤侵犯骨髓，使肝脾γδ T细胞淋巴瘤的病理诊断很难，只能靠特别的染色手段检出。使用抗体或通过流式细胞仪寻找γδ很重要，否则可能诊断错误。

　　Oncology Frontier: Can you outline the treatment options for γδT-cell lymphoma?

　　《肿瘤瞭望》：γδ T细胞淋巴瘤的治疗方案有哪些？

　　Dr Foss: The treatment of γδT-cell lymphoma is still confusing and there is really no good treatment algorithm for these patients. If you look at hepatosplenic T-cell lymphoma， the outcome is poor. Many of those patients are treated with nucleoside analog-based therapies such as pentostatin. If they do respond， many of them will go on to have an allogeneic stem cell transplant which is really the only way to cure them. For the cutaneous γδT-cell lymphomas， they tend to be very aggressive and patients are often treated with various multi-agent chemotherapy regimens. If they respond， then they should progress to an allogeneic stem cell transplant which， again， is really the only way to cure the disease. Unfortunately， we don’t have a lot of data yet looking at some of our newer agents because in the clinical trials， these patients have been excluded. We have learnt from some recent molecular information that STAT inhibitors are possibly relevant here and also the anti-CCR4 antibody will perhaps show some activity in these patients.

　　Foss博士：目前没有很好的γδ T细胞淋巴瘤治疗方案。肝脾γδ T细胞淋巴瘤多采取核苷类似物为基础的方案治疗（如喷司他丁），如果治疗有反应，下一步是异基因造血干细胞移植，这是唯一能治愈的方法。皮肤γδ T细胞淋巴瘤的侵袭性强，常接受多药化疗，如果这种治疗有反应，下一步还是异基因造血干细胞移植，这也是唯一能治愈的方法。不幸的是，目前没有大量新药治疗γδ T细胞淋巴瘤的数据。因为在临床试验这些患者被排除在外。目前来看，Stat抑制剂、抗CCR4抗体药也许会有些治疗效果。