摘要目的 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.

abstractsObjective 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.