18例儿童系统性ALK阳性间变大细胞淋巴瘤的临床特征及患儿预后分析

Clinical Features and Prognostic Factors of 18 Children with Anaplastic Large Cell Lymphoma

目的:分析儿童间变大细胞淋巴瘤(ALCL)的临床特征,探讨治疗效果及预后因素。方法:收集2011年4月至2017年11月期间在福建医科大学附属协和医院小儿血液科收治的18例ALCL的临床、实验室及病理资料进行总结分析。结果:男女比例为2∶1,中位发病年龄6(0.9-11.3)岁,B症状阳性13例,17例首发症状为淋巴结肿大,所有病例伴有多部位受累。4例临床分期Ⅱ期,11例临床分期Ⅲ期,3例Ⅳ期。9例白细胞数增高,CRP>20 mg/L的8例,病理结果均为ALK阳性的间变大细胞淋巴瘤,Ki-67在40%-90%之间。2例在治疗前死亡,1例失访。15例采用CCCG-BNHL-2011方案化疗,中位随访时间41个月,2例早期复发,3年无事件生存率(76.7±10.2)%。Kaplan-Meier生存分析显示,白细胞数增多、CRP水平增高、骨质受累、临床分期对生存的影响具有统计学意义。结论:ALCL是儿童非霍奇金淋巴瘤一个较少见的亚型,疾病的侵袭性强。白细胞数增多、CRP水平增高、骨质受累、临床分期是影响预后的不良因素。

Objective:To analyze the clinical features and to explore the therapeutic efficacy and prognostic factors of children with anaplastic large cell lymphoma(ALCL).Methods:The clinical data of Pediatric Hematology,Union Hospital of Fujian Medical University fr1 o8 children with ALCL admitted in Department of m April w Results:The male to female ratio was 2011 to November 2017 as collected and analyzed.in 2∶1,the median age of onset was,and the B symptom was positive6(0.9-11.3)years old13 cases.The most common initial symptom was lymphadenopathy(in were manifested with multiple organ involvements.17 cases).All patients 4 cases were classified as clinical stageⅡ,11 cases as stageⅢ,and The pathol3 cases as stageⅣ.Laboratory tests revealed ogical results showed all ALK-positive anap9 cases with leukocytosis and lastic large cell lymphoma wit8 cases with CRP>h Ki-20 mg/L.ollow-up time was 67 rate between 40%-90%.The median fapy protoco4 l1 months.of CCCG-2 patients died before treatment,BNHL-1 patient was lost to follow-up.p15 atients accepted chemothersurvival analysis s2 h0 o1 w1.ed2 patients relapsed early,the leukocytosis,increased CRP3 year event-free survival rate was(76.7±10.2)%.Kaplan-Meier level,bone involvement and clinical stage were factors affecting prognosis.Conclusion:ALCL is a relatively rare subtype of childhood non-Hodgkin’s lymphoma with high invasiveness.Leukocytosis,increased CRP level,bone involvement and clinical stage are poor factors affecting the prognosis of patients.