Pillai Vinodh, Tallarico Michael, Bishop Michael R, Lim Megan S
Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA, USA.
Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Br J Haematol. 2016 May;173(4):573-81. doi: 10.1111/bjh.14044. Epub 2016 Mar 15.
Mature T/Natural killer (NK)-cell neoplasms of children and the young adolescent population exhibit higher prevalence in Central and South American and Asian populations and many are associated with Epstein-Barr virus (EBV). They are represented in large part by extranodal T/NK cell lymphomas- nasal-type or extra nasal-type, chronic lymphoproliferative disorders of T/NK cells or chronic active EBV disease, systemic EBV-positive lymphoproliferative disorders of childhood, hydroa vacciniforme-like lymphoma, hepatosplenic T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma among others. Many T/NK cell neoplasms in this age group are derived from cells of the innate immune system, in contrast to adults where they are predominantly from the adaptive immune system. The genetic basis of T/NK cell lymphomas in children and young adolescents remains largely unknown. Anthracycline-based regimens and haematopoietic stem cell transplants (allogeneic and autologous) are current treatment modalities, however it is anticipated that novel targeted therapeutic agents will be available in the near future.
儿童和青少年的成熟T/自然杀伤（NK）细胞肿瘤在中美洲、南美洲和亚洲人群中更为常见，且许多与爱泼斯坦-巴尔病毒（EBV）相关。它们主要包括结外T/NK细胞淋巴瘤——鼻型或鼻外型、T/NK细胞慢性淋巴细胞增殖性疾病或慢性活动性EBV疾病、儿童系统性EBV阳性淋巴细胞增殖性疾病、种痘样水疱病样淋巴瘤、肝脾T细胞淋巴瘤和原发性皮肤γ/δT细胞淋巴瘤等。与成人T/NK细胞肿瘤主要源自适应性免疫系统不同，该年龄组的许多T/NK细胞肿瘤源自先天性免疫系统细胞。儿童和青少年T/NK细胞淋巴瘤的遗传基础在很大程度上仍不清楚。基于蒽环类药物的治疗方案和造血干细胞移植（异基因和自体）是目前的治疗方式，不过预计在不久的将来会有新型靶向治疗药物问世。
