Kobayashi Ryoji, Suzuki Daisuke, Hori Daiki, Kishimoto Kenji, Sano Hirozumi, Nakazawa Atsuko, Yasuda Kazue, Kobayashi Kunihiko
Department of Pediatrics, Sapporo Hokuyu Hospital, Shiroishiku, Sapporo, Japan.
Department of Pathology, National Center for Child Health and Development, Setagaya, Tokyo, Japan.
Pediatr Int. 2015 Oct;57(5):1035-7. doi: 10.1111/ped.12784.
Peripheral T-cell lymphoma (PTCL) is rare in children, and it has a poor prognosis compared with other types of lymphoma. We report the case of a 7-year-old boy with spontaneous improvement of PTCL complicated by hemophagocytic syndrome as the initial symptom. He complained of pain and swelling of the right neck and presented with high fever. Pancytopenia, liver dysfunction, elevated ferritin and soluble interleukin 2 receptor were noted on laboratory tests. Peripheral blood plasma and white blood cells were positive for Epstein-Barr virus (EBV) genome but, after several days, the fever abated and laboratory data improved. On histopathology of lymph node biopsy, he was diagnosed as having PTCL not otherwise specified (PTCL-NOS) with EBV infection. He received no chemotherapy and was disease free at the last follow up, 6 years 8 months after onset. This is probably the first case of spontaneous improvement in PTCL-NOS. Careful treatment planning is therefore necessary in PTCL-NOS, given the possibility of spontaneous improvement of symptoms.
外周T细胞淋巴瘤（PTCL）在儿童中较为罕见，与其他类型的淋巴瘤相比，其预后较差。我们报告了一例7岁男孩，以PTCL并发噬血细胞综合征为首发症状并自发缓解。他主诉右颈部疼痛和肿胀，并伴有高热。实验室检查发现全血细胞减少、肝功能障碍、铁蛋白升高和可溶性白细胞介素2受体升高。外周血血浆和白细胞的爱泼斯坦-巴尔病毒（EBV）基因组呈阳性，但几天后，发热消退，实验室数据有所改善。经淋巴结活检组织病理学检查，他被诊断为未另行特指的PTCL（PTCL-NOS）伴EBV感染。他未接受化疗，在发病后6年8个月的最后一次随访时无疾病。这可能是首例PTCL-NOS自发缓解的病例。因此，鉴于PTCL-NOS症状有自发缓解的可能性，在治疗时需要仔细规划。
