复发/难治性儿童B淋巴母细胞淋巴瘤1例报告及文献复习

Recurrent or refractory childhood B cell lymphoblastic lymphoma:A case report and literature review

目的:探讨B淋巴母细胞淋巴瘤(B-LBL)的临床特点、诊断思路及治疗方法,阐明B-LBL诊疗进展,提高临床医师对该疾病的认识,进一步指导预后评估及治疗。方法:回顾性分析1例儿童B-LBL的临床资料,包括症状、体征、辅助检查、诊断思路、治疗方案、疾病转归,并进行相关文献复习。结果:结合患者临床特点,明确诊断为儿童B-LBL,给予长春新碱+柔红霉素+左旋门冬酰胺+泼尼松方案化疗1周期,同时甲氨蝶呤+地塞米松鞘内注射预防中枢神经性白血病(CNS),25d后骨髓象提示完全缓解(CR)。因患者出现Ⅳ度骨髓抑制,第2疗程更换方案为环磷酰胺+阿糖胞苷+巯基嘌呤,同时给予鞘内注药预防CNS,2周期后再次出现Ⅳ度骨髓抑制,故更换方案为巯基嘌呤+大剂量甲氨蝶呤,同时继续预防CNS,该方案用药2周期末复查骨髓象为CR,第3周期结束后复查骨髓象提示复发(幼稚淋巴细胞10%)。随后多次更换化疗方案,病程中缓解与复发交替,治疗31个月后骨髓象维持稳定CR状态,随后长期口服巯基嘌呤+甲氨蝶呤维持治疗(巯基嘌呤50mg·d-1;甲氨蝶呤25mg,每周1次)至今,现无病生存3年余。结论:B-LBL进展迅速,短期内即可发生骨髓浸润,治疗过程中容易复发,一旦复发即有可能转变为复发/难治性B-LBL。治疗过程中需根据患者耐受程度调整治疗方案,防止化疗不耐受或耐药。早期对B-LBL患者进行危险度分层,做出合理的预后评估并采取及时有效的治疗对改善患者生活质量及延长其生存期至关重要。

Objective:To explore the clinical characteristics,the diagnostic framework,and the treatment methods of B cell lymphoblastic lymphoma(B-LBL),and to clarify the progress of diagnosis and treatment of B-LBL to improve the clinician’s understanding of the disease and provide the guidance for prognostic evaluation and therapeutic options.Methods:The clinical data including symptoms,physical signs,ancillary testings,diagnosis,treatment and disease prognosis of a child suffered from B-LBL were retrospectively analyzed;in the meantime,the relative literatures were reviewed.Results:The patient was definitly diagnosed as B-LBL according to the clinical characteristics and received combination therapy with vincristine,daunorubicin,L-asparaginase,and prednisone as the first course,along with the intrathecal injection of methotrexate and dexamethasone to prevent central nervous system leukemia(CNS-L).The patient achieved complete remission(CR)25 d after the first circle chemotherapy but was diagnosed as degree 4 myelosuppression.Therefore,the second cycle combination therapy was adjusted with cyclophosphamide,cytarabine and 6-MP,and the intrathecal injection to prevent CNS concomitantly.DegreeⅣmyelosuppression appeared repeatedly after 2 cycles and the combination chemotherapy was reajdusted.So mercaptopurine and high dose of methotrexate were given as the 4th cycle,and CNS was prevented continously.The patient kept CR until the second cycle finished but get recurrence after the third chemotherapy(prolymphocytes 10%).Then remission and recurrence were found in the disease counrse during which mary chemotherapy methods were attempted until the patient got stable CR after treatment for 31 months.Then the patient was treated with oral mercaptopurine(50 g·d-1)and methotrexate(25 mg per week)and kept disease-free survival for more than 3 years.Conclusion:B-LBL is a rapidly developed disease with the bone marrow involvement occurring in the short term and easy to relapse during treatment.However,it is extremely easy to transform to recurrent and refractory B-LBL after the first remission.It is of great importance to estimate the risk stratification and to evaluate the prognosis of LBL patients in order to treat as soon as possible for the improvement of one’s life quality and the prolongation of survival.