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

题目: 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. 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. RESULTS: The male to female ratio was 2∶1, the median age of onset was 6 (0.9-11.3) years old, and the B symptom was positive in 13 cases. The most common initial symptom was lymphadenopathy (in 17 cases). All patients were manifested with multiple organ involvements. 4 cases were classified as clinical stage Ⅱ, 11 cases as stage Ⅲ, and 3 cases as stage Ⅳ. Laboratory tests revealed 9 cases with leukocytosis and 8 cases with CRP＞20 mg/L. The pathological results showed all ALK-positive anaplastic large cell lymphoma with Ki-67 rate between 40%-90%. The median follow-up time was 41 months. 2 patients died before treatment, 1 patient was lost to follow-up. 15 patients accepted chemotherapy protocol of CCCG-BNHL-2011. 2 patients relapsed early, the 3 year event-free survival rate was (76.7±10.2)%. Kaplan-Meier survival analysis showed leukocytosis, increased CRP level, bone involvement and clinical stage were factors affecting prognosis. 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.