儿童成熟B细胞非霍奇金淋巴瘤临床特点及预后分析

Clinical features and prognosis of children with mature B-cell non-Hodgkin's lymphoma: an analysis of 28 cases

摘要:

目的 了解儿童成熟B细胞非霍奇金淋巴瘤（B-NHL）的临床特征和治疗结局。方法 28例患儿均以CCCG-B-NHL 2010方案化疗，其中20例联合美罗华治疗，随访31（4~70）个月。回顾性分析患儿的临床特征，以Kaplan-Meier法进行生存分析，并且进行预后因素的单因素分析。结果 28例患儿中伯基特淋巴瘤（BL）17例（61%），弥漫大B细胞型淋巴瘤（DLBCL）8例（29%），3 例（11%）未能进一步分类。首发表现为颈部肿物的13例（46%），颌面部肿物10例（36%），肝脾大9例（32%），腹部包块5例（18%），突眼5例（18%）。LDH<500 U/L者14例，500~1 000 IU/L者3例，≥1 000 IU/L者11例。化疗2疗程后，21例完全缓解、7例部分缓解，截至随访末期，24例持续完全缓解、4例复发。2年无事件生存率为85.7±6.6%。骨髓活检提示骨髓浸润、LDH≥500 IU/L、骨髓肿瘤细胞 > 25%的成熟B-NHL患儿2年累积生存率较低。结论 CCCG-B-NHL 2010方案联合美罗华治疗儿童B-NHL疗效满意，骨髓活检发现的骨髓浸润与不良预后相关。

Abstract:

Objective To study the clinical features and treatment outcome of children with mature B-cell non-Hodgkin's lymphoma (B-NHL). Methods A total of 28 previously untreated children with mature B-NHL were enrolled and given the chemotherapy regimen of CCCG-B-NHL-2010. Among them, 20 were given rituximab in addition to chemotherapy. The children were followed up for 31 months (ranged 4-70 months). A retrospective analysis was performed for the clinical features of these children. The Kaplan-Meier method was used for survival analysis. A univariate analysis was performed to investigate the prognostic factors. Results Among the 28 children, 17 (61%) had Burkitt lymphoma, 8 (29%) had diffuse large B-cell lymphoma (DLBCL), and 3 (11%) had unclassifiable B-cell lymphoma. As for the initial symptom, 13 (46%) had cervical mass, 10 (36%) had maxillofacial mass, 9 (32%) had hepatosplenomegaly, 5 (18%) had abdominal mass, and 5 (18%) had exophthalmos. Of all children, 14 had a lactate dehydrogenase (LDH) level of < 500 IU/L, 3 had a level of 500-1 000 IU/L, and 11 had a level of ≥ 1 000 IU/L. After two courses of chemotherapy, 21 children achieved complete remission and 7 achieved partial remission. At the end of follow-up, 24 achieved continuous complete remission and 4 experienced recurrence. The 2-year event-free survival rate was (85.7±6.6)%. The children with bone marrow infiltration suggested by bone marrow biopsy, serum LDH ≥500 IU/L, and bone marrow tumor cells > 25% had a low 2-year cumulative survival rate. Conclusions The CCCG-B-NHL 2010 chemotherapy regimen combined with rituximab has a satisfactory effect in the treatment of children with B-NHL. Bone marrow infiltration on bone marrow biopsy is associated with poor prognosis.