摘要目的 探讨儿童成熟B细胞淋巴瘤并发急性肿瘤溶解综合征(ATLS)的临床特点.方法 总结患儿临床资料,评估化疗前肿瘤负荷,收集化疗后第8天、3个月及停药前各项评估结果并追踪预后.统计ATLS在B细胞淋巴瘤的发生情况及其与高肿瘤负荷、肾脏侵犯的关系.全部患儿给予大剂量水化碱化、利尿、别嘌呤醇等常规治疗,予血液透析等治疗.结果 (1)103例成熟B细胞淋巴瘤患儿中诊断ATLS者18例(17.5%),病理类型均为伯基特型,18例患儿均为Ⅲ～Ⅳ期且并发巨大瘤块,骨髓幼稚细胞＞25%者7例(38.9%),乳酸脱氢酶≥1000 U/L者11例(61.1%),肾脏侵犯者11例(61.1%);(2)18例(100%)患儿有恶心、呕吐等胃肠道反应;7例(38.9%)发生抽搐(5例为低钙惊厥,2例头颅磁共振提示为双侧枕叶脑白质病变);6例(33.3%)存在严重的少尿或无尿;消化道出血1例(5.6%);(3)全部患儿均有血尿素氮和(或)肌酐升高,高尿酸、高磷及高钾血症、低钙血症;(4)经常规治疗缓解者9例(50.0%),经血液透析和(或)尿酸氧化酶治疗缓解者8例(44.5%);本组接受规律化疗者13例(72.2%),10例至今无事件存活(55.6%),3例停药后复发导致死亡(16.7%);治疗初期放弃治疗或死亡者共5例(27.8%).结论 (1)儿童成熟B细胞淋巴瘤中伯基特型易发生ATLS;(2)高肿瘤负荷、肾脏侵犯是ATLS发生的高危因素;(3)常规应用水化碱化、别嘌呤醇、利尿是治疗ATLS的有效措施,重症者应用血液透析等治疗是有效的缓解手段.

abstractsObjective To investigate risk factors associated with acute tumor lysis syndrome (ATIS) in children with B-cell lymphoma and to explore feasible means for the prophylaxis and treatment.Method Data from 18 children with ATLS in B-cell lymphoma were collected to assess their tumor burden at diagnosis and before chemotherapy. Evaluation was performed at the 8th day, 3 month, and the end of chemotherapy and follow up. The incidence of ATLS in B-cell lymphoma, and the relationship between the incidence of ATLS and whether the kidney was involved and large tumor burden were analyzed respectively.All patients received hydration, alkalinization and received allopurinol routinely. Urate oxidase and hemodialysis treatment were administered in some cases. Result Of the 103 children with B-cell lymphoma, 18 were diagnosed as having ATLS (17.5%). All the 18 cases with ATLS were histopathologically confirmed as having Burkitt's lymphoma. All the patients were at stage Ⅲ or Ⅳ and all had large tumor sizes, and 7 were found to have blasts in the bone marrow ＞ 25% ( 38. 9% ). Lactate dehydrogenase (LDH) levels ≥1000 U/L were found in 11 (61.1%) cases. All patients had developed metabolic abnormalities, including hyperuricemia, hyperphosphatemia, hypocalcemia , and uremia. In terms of clinical features and prognosis, all cases had nausea, vomiting, anorexia, oliguria, and anuria at different levels. One had gastrointestinal bleeding, 7 patients experienced seizures. The etiology in five was hypocalcemia and two had reversible posterior encephalopathy syndrome and all responded well to treatment.Nine cases of ATLS responded to supportive care, 4 required hemodialysis, and the other 4 responded to urate oxidase. Ten cases survived and 8 died. The major cause of death was severe complications and treatment was given up in 5 cases and recurrence occurred in 3 cases. Conclusion ATLS was commonly seen in Burkitt's subtype of B-cell lymphoma. Higher LDH and large tumor sizes and kidney involvement were important risk factors for the development of ATLS in children with B-cell lymphoma. Treatments with hydration, alkalinization and allopurinol were safe and effective. Urate oxidase and hemodialytic treatments should be given timely.