Objective: To investigate the characteristics in pathological diagnosis, clinical features, treatment and prognosis of adult patients with pediatric-type follicular lymphoma (PTFL) . Methods: The clinical and pathological features, laboratory examination, diagnosis and treatment, follow-up results of 5 adult PTFL patients admitted in Jiangsu Province Hospital were retrospectively analyzed, and literature review was conducted in combination with related reports. Results: All 5 patients developed PTFL in their adulthood with a median age of 22 years old (15-33 years) . The initial inanifestation of the disease was local painless lymphadenopathy with no fever, night sweats, emaciation or other systemic B symptoms. Pathological characteristics including typical large follicular structures and high proliferation index were found. Meanwhile, additional clonal rearrangement of immunoglobulin heavy chain gene was observed. However, there was no BCL-2 expression in histochemistry as well as BCL-2 gene abnormality in fluorescence in situ hybridization among these PTFL patients. These adult PTFL patients were all in stage Ⅰ-Ⅱ of the disease. For treatment, they were only treated with local surgical excision after diagnosis while didn't receive subsequent local radiotherapy or systemic immunochemotherapy. During a median follow-up of 27 months, the 5 cases of PTFL kept in a state of sustained complete remission. Conclusion: Adult-onset PTFL is characterized by high pathological proliferation index, while no BCL-2 expression or BCL-2 gene abnormality. Besides, PTFL is clinically manifested as a localized disease that can achieve a quite good prognosis through local surgical intervention. The aforementioned attributes of PTFL are distinctly different from classic adult follicular lymphoma.
目的： 探讨成人儿童型滤泡淋巴瘤（PTFL）患者的病理诊断、临床特征、治疗及转归特点。 方法： 回顾性分析在江苏省人民医院就诊的5例成人PTFL患者的临床病理特点、实验室检查、诊治经过及随访结果，并结合文献进行综述。 结果： 5例PTFL患者均为成年发病，中位年龄22（15~33）岁。起病时表现为局部无痛性淋巴结肿大，无发热、盗汗、消瘦等全身B症状。组织病理表现为典型的大滤泡结构，增殖指数高，存在免疫球蛋白重链基因克隆性重排，组化提示无BCL-2蛋白表达，荧光原位杂交未见BCL-2基因异常。所有患者均为Ⅰ~Ⅱ期，治疗仅通过局部肿块切除，未追加局部放疗或全身系统性免疫化疗。中位随访27个月，5例患者均处于持续缓解状态。 结论： 成人PTFL病理增殖指数较高，无BCL-2表达和基因异常，临床多为局限病变，通过局部手术治疗预后较好，与经典成人滤泡淋巴瘤有显著差异。.
Keywords: Adult; Disease attributes; Pediatric-type follicular lymphoma; Prognosis; Treatment.