在患有 22q11.2 缺失综合征的儿科患者中，表现为可能的γ-δ T 细胞淋巴瘤。
masquerading as possible gamma-delta T-cell lymphoma in a paediatric patient with 22q11.2 deletion syndrome.
机构信息
Department of Paediatrics, Monash Children's Hospital, Clayton, Victoria, Australia
Department of Paediatrics, Monash Children's Hospital, Clayton, Victoria, Australia.
出版信息
BMJ Case Rep. 2021 Nov 30;14(11):e245592. doi: 10.1136/bcr-2021-245592.
A 14-year-old boy with 22q11.2 deletion syndrome and a right ventricular to pulmonary artery xenograft conduit presented to an Australian tertiary children's hospital with prolonged fevers, weight loss, splenomegaly and a high proportion of gamma-delta T cells in peripheral blood and bone marrow, concerning for possible gamma-delta T-cell lymphoma. However, investigations did not reveal evidence of lymphoma or autoimmune disease. After 5 months of intermittent fever episodes and ongoing symptoms, he was found to have an extremely high titre (8192) on serological testing, with the organism also detected on blood PCR. After 6 months of oral azithromycin and rifampicin, with complete resolution of his symptoms 3 months into treatment, his blood PCR was negative and gamma-delta T cells in peripheral blood were decreasing. The titre remained unchanged for some time, but decreased to 2048 around 1 year after treatment was started.
一名 14 岁男孩患有 22q11.2 缺失综合征和右心室至肺动脉异种移植物通道，因持续发热、体重减轻、脾肿大以及外周血和骨髓中大量γ-δ T 细胞而到澳大利亚一家三级儿童医院就诊，考虑可能患有 γ-δ T 细胞淋巴瘤。然而，检查并未发现淋巴瘤或自身免疫性疾病的证据。在经历了 5 个月的间歇性发热发作和持续症状后，他的血清学检测结果显示极高滴度（8192），血液 PCR 也检测到该病原体。在接受 6 个月的口服阿奇霉素和利福平治疗后，治疗 3 个月后症状完全缓解，他的血液 PCR 转为阴性，外周血中的γ-δ T 细胞也在减少。滴度在一段时间内保持不变，但在开始治疗 1 年后降至 2048。
Zotero 插件