Faria S L, Vassallo J, Cosset J M, Brandalise S R
Centro de Investigações Hematológicas Dr. Domingos Boldrini, State University of Campinas, UNICAMP, Brazil.
Med Pediatr Oncol. 1996 Feb;26(2):90-4. doi: 10.1002/(SICI)1096-911X(199602)26:2<90::AID-MPO4>3.0.CO;2-P.
Little clinical information about Hodgkin's disease in children is available from poor countries. The object of this study is to evaluate our data in Campinas, Brazil and hope "to make one dot on the geographic map of this disease more clear."
Between 1978 and 1988, 46 patients under the age of 17 years with biopsy-proven Hodgkin's Disease (HD) were referred for evaluation at Centro Boldrini in Campinas, São Paulo state, in Brazil. Thirty-seven of them were treated and followed-up only at this Center and are the subjects of this analysis. All the original histological slides were obtained, reviewed, and classified according to the Rye system. Staging procedures included exploratory laparotomy in 33 of 37 children, but none had lymphangiography. Treatment was individualized until January 1986 when the German protocol was adopted.
Nineteen cases were classified as nodular sclerosis, 14 as mixed cellularity, and three as lymphocyte depleted. Mean age was 7 years; male/female ratio was 2:1. Fifty percent were advanced stages III and IV and 46% (17/37) had at least one of the systemic B symptoms. Mean follow-up was 81 months (range from 41 to 174 months). Five-year actuarial overall survival was 78%. Two children (5%) had acute myeloid leukemia at 25 and 49 months after diagnosis.
Although distribution of histological subtypes of our cases is similar to other reports in developed countries, as well as percentage of advanced stages III/IV, our patients fared worse when compared to those reports. The reason for this continues to remain unclear but it does not seem to be related to histology subtypes.
贫困国家关于儿童霍奇金病的临床信息较少。本研究的目的是评估我们在巴西坎皮纳斯的数据，希望“使该疾病地理分布图上的一个点更加清晰”。
1978年至1988年期间，46例17岁以下经活检证实为霍奇金病（HD）的患者被转诊至巴西圣保罗州坎皮纳斯的博尔迪尼中心进行评估。其中37例仅在该中心接受治疗和随访，是本分析的对象。所有原始组织学切片均已获取、复查并根据赖氏系统进行分类。分期程序包括对37名儿童中的33名进行剖腹探查，但均未进行淋巴管造影。1986年1月采用德国方案之前，治疗是个体化的。
19例被分类为结节硬化型，14例为混合细胞型，3例为淋巴细胞消减型。平均年龄为7岁；男女比例为2:1。50%为III期和IV期晚期，46%（17/37）至少有一项全身B症状。平均随访时间为81个月（范围为41至174个月）。五年精算总生存率为78%。两名儿童（5%）在诊断后25个月和49个月患急性髓细胞白血病。
尽管我们病例的组织学亚型分布与发达国家的其他报告相似，III/IV期晚期的比例也相似，但与那些报告相比，我们的患者预后更差。其原因仍不清楚，但似乎与组织学亚型无关。
