中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2008年
3期
209-214
,共6页
张永红%段颜龙%杨菁%金玲%周春菊%高子分
張永紅%段顏龍%楊菁%金玲%週春菊%高子分
장영홍%단안룡%양정%금령%주춘국%고자분
淋巴瘤,非霍奇金氏%伯基特淋巴瘤%儿童%药物疗法,联合
淋巴瘤,非霍奇金氏%伯基特淋巴瘤%兒童%藥物療法,聯閤
림파류,비곽기금씨%백기특림파류%인동%약물요법,연합
Lymphoma,non-Hogkins%Burkitt lymphoma%Child%Drug therapy,combination
目的 认识儿童伯基特淋巴瘤(BL)和伯基特样淋巴瘤(BLL)的病理及临床特点,总结大剂量、短疗程化疗方案的疗效,探讨治疗相关的合并症及预后影响因素.方法 2003年2月-2006年4月住院治疗的淋巴瘤患儿40例,全部行活检病理形态及免疫组化检查,并按WHO新的病理分型标准进行分型,参照儿童非霍奇金淋巴瘤(NHL)的St.Jude分期标准进行分期.采用大剂量、短疗程的改良LMB89方案进行治疗.根据不同分期、不同危险因素及对治疗反应的评估结果,将患儿分成A、B、c三个治疗组.结果 临床特点:发病年龄2~14岁,中位发病年龄6.9岁;男33例,女7例,男:女=4.7:1;腹腔肿物及急腹症为最常见表现.病理示BL 30例、BLL 10例;7例(17.5%)在瘤组织中检测到EBER-1阳性细胞,19例血清中EB病毒抗体阳性.确诊时I~Ⅱ期患儿9例,Ⅲ~Ⅵ期患儿31例;伴中枢侵犯者3例,骨髓侵犯者2例.疗程2~8个月.随访率100%,中位追踪时间22.6个月.至2006年12月底,实际存活35例,1年生存率88.7%,预计3年无进展生存率81.8%.化疗中Ⅲ~Ⅳ度骨髓抑制的发生率在B组和C组分别为97.5%和100%,3例出现重症黏膜炎.5例死亡,其中2例为副作用相关死亡.单因素分析显示,临床分期为Ⅳ期、治疗3个月时评估有残留病灶、病程小于15 d与预后不良相关(P<0.05).结论 采用大剂量、短疗程方案治疗BL和BLL疗效好,预计3年无进展生存率81.8%.病史小于15 d、诊断时为Ⅳ期、治疗3个月未达完全缓解者与预后不良相关.
目的 認識兒童伯基特淋巴瘤(BL)和伯基特樣淋巴瘤(BLL)的病理及臨床特點,總結大劑量、短療程化療方案的療效,探討治療相關的閤併癥及預後影響因素.方法 2003年2月-2006年4月住院治療的淋巴瘤患兒40例,全部行活檢病理形態及免疫組化檢查,併按WHO新的病理分型標準進行分型,參照兒童非霍奇金淋巴瘤(NHL)的St.Jude分期標準進行分期.採用大劑量、短療程的改良LMB89方案進行治療.根據不同分期、不同危險因素及對治療反應的評估結果,將患兒分成A、B、c三箇治療組.結果 臨床特點:髮病年齡2~14歲,中位髮病年齡6.9歲;男33例,女7例,男:女=4.7:1;腹腔腫物及急腹癥為最常見錶現.病理示BL 30例、BLL 10例;7例(17.5%)在瘤組織中檢測到EBER-1暘性細胞,19例血清中EB病毒抗體暘性.確診時I~Ⅱ期患兒9例,Ⅲ~Ⅵ期患兒31例;伴中樞侵犯者3例,骨髓侵犯者2例.療程2~8箇月.隨訪率100%,中位追蹤時間22.6箇月.至2006年12月底,實際存活35例,1年生存率88.7%,預計3年無進展生存率81.8%.化療中Ⅲ~Ⅳ度骨髓抑製的髮生率在B組和C組分彆為97.5%和100%,3例齣現重癥黏膜炎.5例死亡,其中2例為副作用相關死亡.單因素分析顯示,臨床分期為Ⅳ期、治療3箇月時評估有殘留病竈、病程小于15 d與預後不良相關(P<0.05).結論 採用大劑量、短療程方案治療BL和BLL療效好,預計3年無進展生存率81.8%.病史小于15 d、診斷時為Ⅳ期、治療3箇月未達完全緩解者與預後不良相關.
목적 인식인동백기특림파류(BL)화백기특양림파류(BLL)적병리급림상특점,총결대제량、단료정화료방안적료효,탐토치료상관적합병증급예후영향인소.방법 2003년2월-2006년4월주원치료적림파류환인40례,전부행활검병리형태급면역조화검사,병안WHO신적병리분형표준진행분형,삼조인동비곽기금림파류(NHL)적St.Jude분기표준진행분기.채용대제량、단료정적개량LMB89방안진행치료.근거불동분기、불동위험인소급대치료반응적평고결과,장환인분성A、B、c삼개치료조.결과 림상특점:발병년령2~14세,중위발병년령6.9세;남33례,녀7례,남:녀=4.7:1;복강종물급급복증위최상견표현.병리시BL 30례、BLL 10례;7례(17.5%)재류조직중검측도EBER-1양성세포,19례혈청중EB병독항체양성.학진시I~Ⅱ기환인9례,Ⅲ~Ⅵ기환인31례;반중추침범자3례,골수침범자2례.료정2~8개월.수방솔100%,중위추종시간22.6개월.지2006년12월저,실제존활35례,1년생존솔88.7%,예계3년무진전생존솔81.8%.화료중Ⅲ~Ⅳ도골수억제적발생솔재B조화C조분별위97.5%화100%,3례출현중증점막염.5례사망,기중2례위부작용상관사망.단인소분석현시,림상분기위Ⅳ기、치료3개월시평고유잔류병조、병정소우15 d여예후불량상관(P<0.05).결론 채용대제량、단료정방안치료BL화BLL료효호,예계3년무진전생존솔81.8%.병사소우15 d、진단시위Ⅳ기、치료3개월미체완전완해자여예후불량상관.
Objective To summarize the histological and clinical characteristics of 40 cases with Burkitt's and Burkitt-like lymphoma in children,to evaluate the effects of treatment with international regimen,and to explore the treatment-related complications and prognostic factors.Methods Forty patients with Burkitt's and Burkitt-like lymphoma were registered in Beijing Children Hospital from Feb 2003 to Apr 2006.The diagnosis was confirmed by histology and immunohistochemistry of biopsy,and clinical staging by the examination of imaging,cerebrospinal fluid and bone marrow based on St.Jude system.Intensive,shortterm chemotherapy witch was modified from LMB89 protocol was given to the patients.Results Of the 40 patients,30 were diagnosed as Burkitt's lymphoma (BL) and 10 as Burkitt-like lymphoma (BLL).Antibody against Epstein-Barr virus (EBV-Ab) was positive in 19 cases at diagnosis,only 7 of the patients were positive for EBER.Thirty-three of the cases were male and 7 female(M:F=4.7:1);the median age was 6 years 9 months.The most frequently seen clinical characteristics were abdominal masses and surgical abdomen.Nine cases were at stage Ⅰ-Ⅱ and 31 cases at stage Ⅲ-Ⅳ at diagnosis:CNS was involved in 4 cases and bone marrow in 2 cases.The courses of treatment were approximately 2-8 months.All the patients were followed up,the median follow-up period was 22.6 months.After chemotherapy,35 patients (88.7%)were still alive during the one-year follow-up.The 3-year event-free survival (EFS) rate was 81.8%.Major toxicity was myelosuppression and mucositis.Stage Ⅲ to Ⅳ of myelosuppression occurred in the most patients with unresected tumor and CNS-involvement.Of 5 patients who died,2 died of infection,2 died of lymphoma progression during chemotherapy.and 1 died of relapse.Conclusion Burkitt's and Burkitt-like lymphoma are the most common NHL in children with rapid clinical process.Outcome was greatly improved by current intensive,short-term chemotherapy regimen,the 3-year EFS was 81.8% including the patients who were in advanced stage.Childhood lymphoma with short clinical history,stage Ⅳ and residual disease after 3 months of therapy are associated with poor prognosis.