PDF(Pubmed)
Abstract：
BACKGROUND: Angioimmunoblastic T-cell lymphoma is an uncommon subtype of peripheral T-cell lymphoma in children with fewer than 20 cases reported in literature.
METHODS: A 3-year-old Omani boy was diagnosed with ataxia-talengectasia presenting with fever and generalized lymphadenopathy. His biopsy revealed atypical lymphocytic infiltrate consistent with the diagnosis of angioimmunoblastic T-cell lymphoma. Within 3 weeks from the initial presentation and without any neoadjuvant therapy, he showed complete recovery of symptoms with absence of fever and regression of all previously affected lymph nodes. He has remained in remission ever since.
CONCLUSIONS: This is the first report of spontaneous improvement of angioimmunoblastic T-cell lymphoma in a patient with ataxia-telangiectasia who was 3 years old at presentation. Owing to the paucity of similar cases, this report adds valuable diagnostic, therapeutic, and monitoring data.
METHODS: A 3-year-old Omani boy was diagnosed with ataxia-talengectasia presenting with fever and generalized lymphadenopathy. His biopsy revealed atypical lymphocytic infiltrate consistent with the diagnosis of angioimmunoblastic T-cell lymphoma. Within 3 weeks from the initial presentation and without any neoadjuvant therapy, he showed complete recovery of symptoms with absence of fever and regression of all previously affected lymph nodes. He has remained in remission ever since.
CONCLUSIONS: This is the first report of spontaneous improvement of angioimmunoblastic T-cell lymphoma in a patient with ataxia-telangiectasia who was 3 years old at presentation. Owing to the paucity of similar cases, this report adds valuable diagnostic, therapeutic, and monitoring data.
摘要：
背景：血管免疫母细胞性T细胞淋巴瘤是一种罕见的儿童外周T细胞淋巴瘤亚型，文献报道病例少于20例。
方法：一名3岁的阿曼男孩被诊断为共济失调性扩张症，表现为发热和全身淋巴结肿大。他的活检显示非典型淋巴细胞浸润与血管免疫母细胞性T细胞淋巴瘤的诊断一致。在初次就诊后3周内，没有任何新辅助治疗，他的症状完全恢复，没有发烧，所有以前受影响的淋巴结消退。从那以后，他一直处于缓解状态。
结论：这是首例3岁的共济失调性毛细血管扩张症患者血管免疫母细胞性T细胞淋巴瘤自发改善的报道。由于类似案件很少，这份报告增加了有价值的诊断，治疗性的,和监测数据。
方法：一名3岁的阿曼男孩被诊断为共济失调性扩张症，表现为发热和全身淋巴结肿大。他的活检显示非典型淋巴细胞浸润与血管免疫母细胞性T细胞淋巴瘤的诊断一致。在初次就诊后3周内，没有任何新辅助治疗，他的症状完全恢复，没有发烧，所有以前受影响的淋巴结消退。从那以后，他一直处于缓解状态。
结论：这是首例3岁的共济失调性毛细血管扩张症患者血管免疫母细胞性T细胞淋巴瘤自发改善的报道。由于类似案件很少，这份报告增加了有价值的诊断，治疗性的,和监测数据。
