发生在儿童及成人的儿童型滤泡性淋巴瘤的临床病理分析

Clinicopathological observation of pediatric-type follicular lymphoma occurring in children and adults: a report of three case.

目的探讨发生在儿童及成人的儿童型滤泡性淋巴瘤的临床病例特点。方法对2例典型儿童滤泡性淋巴瘤和1例成人的儿童型滤泡性淋巴瘤进行形态学、免疫组化及克隆性分析，并随访4~18个月。结果3例患者均为男性，发病年龄分别为9岁、14岁及51岁。病变具有儿童滤泡淋巴瘤的典型形态学及临床表现，包括形态上呈现大而扩张的滤泡，显著的满天星现象，组织学呈高级别，具有高的增殖指数，多聚酶链分析显示免疫球蛋白基因单克隆性；病变处于临床Ⅰ期，2例儿童患者经治疗后无复发，1例成人患者未治疗，随访无进展。结论儿童和成人均可发生儿童型FL，具有类似的临床病理特点，预后良好。

Objective To investigate clinicopathological features of pediatric - type follicular lymphoma occurring in children and adults. Methods Pediatric - type follicular lymphoma occurring in two children and one adult were analyzed by light microscopy,immunohistochemistry and polymerase chain reaction（PCR）analysis. These patients were followed for 4 ~ 18 months. Results These three patients included 2 boys and 1 male with the ages of 9,14 and 51. All cases had classic morphologic features of pediatric - type follicular lymphoma,including large,expansile follicles,＂starry - sky＂ pattern,and a high histological grade. Clonal immunoglobulin gene rearrangements were demonstrated by PCR analysis. All patients showed a low - stage disease with excellent outcome. Two pediatric patients received chemotherapy,achieved complete remission, and experienced no relapses. The untreated adult patient that lacked Bcl -2 rearrangement did not progress. Conclusion Pediatric - type FL can occur in both children and adults with similar morphologic,clinical features and a favorable prognosis.