Department of Pediatric Hematology and Oncology, The National Oncology Centre, Royal Hospital, Ministry of Health, Muscat, Oman.
Department of Pathology, Royal Hospital, Ministry of Health, Muscat, Oman.
J Med Case Rep. 2023 Aug 4;17(1):347. doi: 10.1186/s13256-023-04050-5.
Angioimmunoblastic T-cell lymphoma is an uncommon subtype of peripheral T-cell lymphoma in children with fewer than 20 cases reported in literature.
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.
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.
血管免疫母细胞性 T 细胞淋巴瘤是儿童外周 T 细胞淋巴瘤的一种罕见亚型，文献中报道的病例少于 20 例。
一名 3 岁的阿曼男孩被诊断为共济失调毛细血管扩张症，表现为发热和全身淋巴结病。他的活检显示非典型淋巴细胞浸润，符合血管免疫母细胞性 T 细胞淋巴瘤的诊断。从初次就诊到 3 周内，他没有接受任何新辅助治疗，症状完全缓解，没有发热，所有先前受累的淋巴结均消退。此后他一直处于缓解状态。
这是首例报道的在 presentation 时为 3 岁的共济失调毛细血管扩张症患者中出现的血管免疫母细胞性 T 细胞淋巴瘤自发改善的病例。由于类似病例较少，因此该报告提供了有价值的诊断、治疗和监测数据。
