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在马拉维,接受环磷酰胺单一疗法的伯基特淋巴瘤患儿的长期生存情况。

Long term survival of children with Burkitt lymphoma in Malawi after cyclophosphamide monotherapy.

作者信息

Kazembe P, Hesseling P B, Griffin B E, Lampert I, Wessels G

机构信息

Lilongwe Central Hospital, Malawi.

出版信息

Med Pediatr Oncol. 2003 Jan;40(1):23-5. doi: 10.1002/mpo.10190.

Abstract

BACKGROUND

Between 1991 and 1997, limited funding at times restricted available treatment for children with Burkitt lymphoma (BL) to cyclophosphamide (CPM) monotherapy at Lilongwe Central Hospital, Malawi. Our objective was to assess long-term survival in Malawian children with Burkitt lymphoma (BL) who had received one or more treatments with intravenous CPM at 40 mg/kg/dose at 14-day intervals.

PROCEDURE AND RESULTS

The study population consisted of 92 children in whom BL had been confirmed on fine needle aspirates (FNA), a home address had been documented on discharge from hospital, and the treatment records could be verified. Only the clinical site(s) of disease had been recorded. The M:F ratio was 1.4 and median age 8 years. A clinical officer on motorcycle attempted to locate the given addresses and interview parents or other sources. In 19 patients, the address was incorrect. Of 73 evaluable patients, 40 children are alive at a mean follow-up time of 59 (range: 29-104) months. The survival rate was 63.5% in 52 children with BL of the head only, and 33.3% in 21 children with primary disease involving the abdomen or other sites. Survivors had received a median number of 6 (range: 1-12), non-survivors 4 (range: 1-12), and untracable children 3 (range: 1-11) courses of CPM.

CONCLUSIONS

We confirmed that CPM could cure children with facial and abdominal BL. The unavoidable bias in the selection of patients and the variable amount of CPM given, precludes accurate survival estimates. A prospective study with proper staging, assessment of FNA, marrow and cerebrospinal fluid with modern techniques, a standard treatment protocol, and adequate follow-up will better define the current therapeutic value of CPM monotherapy. CPM can be purchased at about 3 US dollars per 500 mg.

摘要

背景

1991年至1997年期间,资金有限,马拉维利隆圭中心医院有时只能用环磷酰胺(CPM)单药疗法治疗伯基特淋巴瘤(BL)患儿。我们的目的是评估在马拉维接受过一次或多次静脉注射CPM(剂量为40mg/kg,每14天一次)治疗的伯基特淋巴瘤(BL)患儿的长期生存率。

程序与结果

研究人群包括92名经细针穿刺抽吸活检(FNA)确诊为BL、出院时记录了家庭住址且治疗记录可核实的儿童。仅记录了疾病的临床部位。男女比例为1.4,中位年龄8岁。一名骑摩托车的临床工作人员试图找到给定的地址并采访家长或其他信息来源。19名患者的地址有误。在73名可评估的患者中,40名儿童存活,平均随访时间为59个月(范围:29 - 104个月)。仅头部患BL的52名儿童的生存率为63.5%,原发性疾病累及腹部或其他部位的21名儿童的生存率为33.3%。存活者接受CPM治疗的中位数疗程为6个(范围:1 - 12个),非存活者为4个(范围:1 - 12个),无法追踪的儿童为3个(范围:1 - 11个)。

结论

我们证实CPM可治愈面部和腹部BL患儿。患者选择中不可避免的偏差以及给予的CPM量不同,妨碍了准确的生存估计。一项采用现代技术进行适当分期、评估FNA、骨髓和脑脊液、标准治疗方案及充分随访的前瞻性研究,将能更好地确定CPM单药疗法目前的治疗价值。每500mg CPM的售价约为3美元。

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