Schellong G, Riepenhausen M
University Children's Hospital Münster, Department of Paediatric Haematology and Oncology, Münster.
Klin Padiatr. 2004 Nov-Dec;216(6):364-9. doi: 10.1055/s-2004-832340.
For a long time, a main focus of paediatric therapy studies for Hodgkin's disease (HD) has been on diminishing adverse late effects. Consequently, the long-term follow-up of patients after HD is very important. The HD late effects project of the GPOH, which evolved from 5 consecutive German-Austrian DAL therapy studies, was aimed at establishing a basis for the further improvement of therapy concepts and of long-term surveillance.
The original cohort consisted of 1 245 study patients from 92 centres enrolled in the DAL studies HD-78 to HD-90 between 1978 and 1995. Initially, follow-up data were submitted by the participating study centres. When the majority of the patients had reached adult age and were no longer seen by the originally treating paediatric colleagues, we contacted them directly by mail every 2-3 years. At the time of analysis (March 2004) information from the preceding 6 years was available in 78.6 % of the patients alive.
The median follow-up period of patients at the date of last information was 11.1 years (max. 25.5 years), the median age was 23.7 years (max. 41.1 years). The present report is focused on three out of a wide range of problems evaluated, namely cause of death in 14 patients expired after 10-21 years' follow-up, overwhelming post-splenectomy infections (18 events, 11 fatal), and 46 secondary malignancies. The OS rate after 24 years is 87 % (SE 3 %) in the total group, 83 % (SE 3 %) in 335 asplenic patients, and 93 % (SE 2 %) in 910 non- or partially splenectomised patients. We have initiated activities to improve the prophylactic measures against overwhelming infections in this risk group of asplenic patients. The cumulative incidences of secondary malignancies (SM) after 22 years is 11 % (SE 2 %) for all SM, 10 % (SE 2 %) for solid tumours, 0.8 % (SE 0.5 %) for NHL, and 0.6 % (SE 0.3 %) for leukaemias. The cumulative incidence of breast cancer in female patients at the age of 35 years is 4.0 % (SE 2 %). The effect of reducing the radiotherapy doses in the studies HD-87/HD-90 will become evident within the next years.
长期以来，霍奇金淋巴瘤（HD）儿科治疗研究的一个主要重点是减少不良晚期效应。因此，HD患者的长期随访非常重要。德国儿科肿瘤学协会（GPOH）的HD晚期效应项目源自5项连续的德奥DAL治疗研究，旨在为进一步改进治疗理念和长期监测奠定基础。
原始队列由1978年至1995年间参与DAL研究HD - 78至HD - 90的92个中心的1245名研究患者组成。最初，随访数据由参与研究的中心提交。当大多数患者达到成年年龄且最初治疗的儿科同事不再诊治他们时，我们每2 - 3年直接通过邮件与他们联系。在分析时（2004年3月），78.6%存活患者可获得前6年的信息。
在最后一次信息记录时，患者的中位随访期为11.1年（最长25.5年），中位年龄为23.7岁（最长41.1岁）。本报告聚焦于评估的众多问题中的三个，即14例患者在随访10 - 21年后死亡的原因、脾切除术后严重感染（18例事件，11例死亡）以及46例继发恶性肿瘤。24年后，全组的总生存率（OS）为87%（标准误3%），335例无脾患者为83%（标准误3%），910例未行或部分脾切除患者为93%（标准误2%）。我们已开展活动，以改进针对无脾患者这一风险群体的严重感染预防措施。22年后继发恶性肿瘤（SM）的累积发生率，所有SM为11%（标准误2%），实体瘤为10%（标准误2%），非霍奇金淋巴瘤（NHL）为0.8%（标准误0.5%），白血病为0.6%（标准误0.3%）。35岁女性患者乳腺癌的累积发生率为4.0%（标准误2%）。HD - 87/HD - 90研究中降低放疗剂量的效果将在未来几年显现。
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