儿童原发性骨淋巴瘤11例并文献复习

Primary bone lymphoma in children:report of 11 cases and review of literature

目的探讨儿童原发性骨淋巴瘤的临床表现、病理类型、治疗及预后等。方法回顾性分析首都医科大学附属北京儿童医院2016年1月至2020年1月收治的11例初诊为原发性骨淋巴瘤患儿的临床资料,包括性别、发病年龄、原发受累部位、临床分期、病理类型、是否骨折及临床转归等,并复习相关文献。结果11例患儿均为初治原发性骨淋巴瘤,中位发病年龄8.6岁(2.7~12.3岁),其中男性7例,女性4例;弥漫大B细胞淋巴瘤(DLBCL)7例,B淋巴母细胞淋巴瘤(BLL)3例,间变性大细胞淋巴瘤(ALCL)1例;首发症状为骨痛8例,局部肿胀1例,跛行1例,发热1例;临床分期Ⅰ期1例,Ⅱ期7例,Ⅳ期3例;最常见的受累部位为股骨、胫骨。所有患儿均依据不同病理类型接受化疗,中位随访时间45个月(7~80个月)。10例完全缓解,1例BLL患儿化疗缓解后因骨髓复发死亡。结论儿童原发性骨淋巴瘤临床表现隐匿,以DLBCL多见,接受规范治疗预后良好。

Objective To investigate the clinical manifestations,pathological features,treatment and prognosis of primary bone lymphoma in children.Methods The clinical data of children who were initially diagnosed as primary bone lymphoma and treated in Beijing Children's Hospital Affiliated to Capital Medical University from January 2016 to January 2020 were retrospectively analyzed,including gender,onset age,primary involvement site,clinical stage,pathological type,fracture,and clinical outcome.The related literature was reviewed.Results All 1l children were initially diagnosed as primary bone lymphoma,with a median age of onset of 8.6 years old(2.7-12.3 years old),including 7 males and 4 females.There were 7 cases of diffuse large B-cell lymphoma(DLBCL),3 cases of B lymphoblastic lymphoma(BLL),and 1 case of anaplastic large cell lymphoma(ALCL).The initial symptoms were bone pain in 8 cases,local swelling in 1 case,limp in 1 case,and fever in 1 case.One case was in stage I,7 cases were in stage I,and 3 cases were in stage IV,and the most common sites of involvement were femur and tibia.All 1l cases were treated with chemotherapy according to diferent pathological types,with a median follow-up time of 45 months(7-80 months).Ten cases got complete remission,1 case of BLL died of bone marrow recurrence after chemotherapy remission.Conclusions The clinical manifestations of primary bone lymphoma in children are insidious,DLBCL is the most common pathological type,and the prognosis is good after standardized treatment.