儿童少见类型非霍奇金淋巴瘤14例

Clinical analysis of 14 cases of rare non - Hodgkin lymphoma in children

目的分析几类少见儿童非霍奇金淋巴瘤（NHL）的临床特点和预后，探讨相关治疗进展。方法回顾性分析上海交通大学医学院附属上海儿童医学中心2004年1月至2013年12月10年间收治的14例少见类型NHL患儿的临床资料，讨论其临床特点、治疗方法与预后。结果皮下脂膜炎样T细胞淋巴瘤（SPTCL）6例，痘疱样淋巴瘤（HVLL）3例，儿童滤泡性淋巴瘤（PFL）2例，结外NK／T细胞淋巴瘤鼻型（ENKTL）3例。71．4％（10／14例）患儿以皮肤病变起病，病程较长（发病至确诊中位时间10个月），71．4％（10／14例）患儿伴发热，50．0％（7／14例）出现肝脾大，本组病例无中枢神经系统及骨髓浸润，外周血≥二系异常占28．6％（4／14例）。本组主要采用CHOP（环磷酰胺＋多柔比星＋长春新碱＋泼尼松）或成熟B细胞性NHL化疗方案，50．0％（7／14例）病例加用干扰素治疗，1例复发患儿再次化疗后行异基因造血干细胞移植。初治完全缓解71．4％（10／14例），未失访生存者中位随访时间26个月（12～64个月），死亡2例，失访3例，复发1例。3年总生存率及无事件生存率分别为0．84、0．57。结论儿童少见类型NHL早期临床表现不典型，中枢／骨髓浸润发生率低，发病至确诊时间长，病理诊断较困难，常用临床分期标准不适用，目前尚无标准治疗方案，运用成熟B细胞性NHL化疗方案，并加用干扰素治疗，SPTCL、HVLL及PFL预后相对良好。

Objective To analyze the clinical characteristics and prognosis of 4 rare types of non - Hodgkin lymphoma（NHL） in children, and to discuss the progress in treatment. Methods Clinical data of 14 patients with rare types of NHL at Shanghai Children＇s Medical Center, Shanghai Jiaotong University School of Medicine between January 2004 and December 2014 were retrospectively analyzed, and their clinical features, treatment and prognosis were dis- cussed. Results Fourteen cases were reported including 6 subcutaneous panniculitis - like T - cell lymphoma （SPTCL） ,3 hydroa vaeciniforme - like cutaneous lymphoma（HVLL） ,2 pediatric follicular lymphoma（PFL） and 3 ex- tranodal NK/T -cell lymphoma,and nasal type（ ENKTL）. Ten patients （71.4%） primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed （the median was 10 months） ,71.4% （10/14 cases） of them associated with fever and 50.0% （7/14 cases） with liver and spleen enlargement, and no evidence of central nervous system （CNS） and bone marrow （BM） involvement was observed,while 28.6% patients （4/14 cases） had more than two lines＇ abnormalities in peripheral blood examination. Since there were no standard treatment guide- lines, most patients received CHOP （ Cyelophosphamide ＋ Adriamycin ＋ Vineristine ＋ Prednisone） and/or mature B - cell NHL -like therapy, and 50.0% （7/14 cases） of them received interferon therapy in addition, while 1 patient re- ceived allogeneic hematopoietic stem cell transplantation after recurrence. The complete remission was achieved in 71.4% （10/14 cases） of all the patients. Two of them died,3 lost follow- up ,and 1 relapsed. The 3 -year overall survi- val and event free survival rates were 0.84 and 0.57, respectively, after a median follow - up of 26 months （ range 12 - 64 months ）. Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS/BM infil- tration and long course. It is hard to make pathological diagnosis and differentiation. It is also inappropriate to apply the commonly used staging system to these rare types of NHL. No standard treatment has been found by now. SPTCL, HVLL and PFL have relatively good outcomes when treated with mature B - cell NHL - type therapy plus interferon therapy.