PDF(Pubmed)
Abstract：
Non-Hodgkin lymphoma (NHL) is among the five most common pediatric cancer diagnoses in children and adolescents and consists of a heterogeneous group of lymphoid tissue malignancies -with B-cell-derived NHL accounting for nearly 80% of cases. Novel and high-throughput diagnostic tools have significantly increased our understanding of B-NHL biology and molecular pathogenesis, leading to new NHL classifications and treatment options. This retrospective cohort study investigated 17 cases of both mature B-cell NHL (Burkitt lymphoma or BL; Diffuse large B-cell lymphoma or DLBCL; Primary mediastinal large B-cell lymphoma or PMBCL; Follicular lymphoma or FL) and immature B-cell progenitor NHL (B-lymphoblastic lymphoma or BLL) that were treated in a tertiary Pediatric Hematology-Oncology Department during the last 20 years. Modern NHL protocols for children, adolescents, and young adults, along with the addition of rituximab, are safe and efficient (100% overall survival; one relapse). Elevated ESR was more prevalent than elevated LDH. Analyses have focused on immune reconstitution (grade ≥3 infections, lymphocyte and immunoglobulin levels recovery) and body-mass-index changes post-treatment, late effects (in 53% of patients), and the presence of histology markers BCL2, BCL6, CD30, cMYC, and Ki-67%. One patient was diagnosed with a second malignant neoplasm (papillary thyroid cancer).
摘要：
非霍奇金淋巴瘤（NHL）是儿童和青少年中五种最常见的儿科癌症诊断之一，由一组异质性的淋巴组织恶性肿瘤组成-B细胞衍生的NHL占病例的近80％。新的高通量诊断工具显著增加了我们对B-NHL生物学和分子发病机制的理解。导致新的NHL分类和治疗选择。这项回顾性队列研究调查了17例成熟的B细胞NHL（伯基特淋巴瘤或BL；弥漫性大B细胞淋巴瘤或DLBCL；原发性纵隔大B细胞淋巴瘤或PMBCL；滤泡性淋巴瘤或FL）和未成熟的B细胞祖细胞NHL（B淋巴母细胞淋巴瘤或BLL），在过去20年中在三级小儿血液肿瘤科接受治疗。现代儿童NHL协议，青少年,和年轻人，随着利妥昔单抗的加入，是安全和有效的（100％的总生存率；一次复发）。ESR升高比LDH升高更普遍。分析集中在免疫重建(≥3级感染，淋巴细胞和免疫球蛋白水平恢复）和治疗后体重指数变化，晚期影响（在53％的患者中），和组织学标记BCL2，BCL6，CD30，cMYC的存在，Ki-67%。一名患者被诊断患有第二恶性肿瘤（甲状腺乳头状癌）。
