Bień Ewa, Stachowicz-Stencel Teresa, Zawitkowska-Klaczyńska Joanna, Adamkiewicz-Drozyńska Elzbieta, Odój Teresa, Połczyńska Katarzyna, Mitura-Lesiuk Małgorzata, Stefanowicz Joanna, Sierota Danuta, Szołkiewicz Anna, Birkholz Dorota, Hennig Marcin, Kowalczyk Jerzy R, Balcerska Anna
Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii, Akademia Medyczna, ul. Debinki 7, 80-211 Gdańsk, Poland.
Med Wieku Rozwoj. 2006 Jul-Sep;10(3 Pt 1):631-8.
The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease.
The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh).
Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopathological type of HD predominated. Poor response to standard treatment was observed in five children. One patient received additional cycles of chemotherapy MVPP/B-DOPA, four children were administered the 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd therapy lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation. Radiotherapy was given to 13 children. 13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years. In two of them late complications affecting hormonal status, cardio-pulmonary disorders and chronic B and C hepatitis were observed. Two children died including one admitted in a very severe condition, after long-lasting medical history who died of neutropenia-related sepsis. The second boy died 12 months after stem cell transplantation because of a second neoplasm--acute myeloblastic leukaemia.
Chemo- and radiotherapy implemented according to protocols approved by the PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis made at earlier stages would result in giving less aggressive therapy, connected with a lower risk of durable late complications.
目前，霍奇金淋巴瘤（HD）患儿的治愈率超过90%，但IV期HD的预后要差得多。本研究的目的是分析IV期霍奇金淋巴瘤患儿的初始症状、病程及肿瘤治疗结果。
分析的队列包括15例IV期HD患儿（男/女：11/4，平均年龄：12岁），于1993年1月至2005年3月在波兰格但斯克和卢布林的两个儿科肿瘤中心接受治疗。诊断和治疗按照波兰儿科白血病/淋巴瘤研究组（PPGBCh）批准的现行方案进行。
初始症状的平均持续时间为4.5个月，大多数患儿表现出HD的全身症状。诊断时，9例患儿发现纵隔和/或肺门淋巴结受累，6例有肺部浸润，5例、3例和1例患儿分别有脾脏、肝脏和骨骼受累。HD的结节硬化组织病理学类型占主导。5例患儿对标准治疗反应不佳。1例患儿接受了额外疗程的化疗MVPP/B - DOPA，4例患儿接受了二线化疗挽救方案95。1例对一线和二线治疗反应极差的男孩额外接受了大剂量化疗及外周血干细胞移植。13例患儿接受了放疗。15例患儿中有13例存活且无疾病，平均随访时间为6年。其中2例出现了影响激素状态、心肺功能障碍和慢性B型及C型肝炎的晚期并发症。2例患儿死亡，其中1例病情非常严重，在长期病史后死于中性粒细胞减少相关的败血症。第二个男孩在干细胞移植12个月后死于第二种肿瘤——急性髓细胞性白血病。
按照PPGBCh批准的方案对IV期HD患儿实施化疗和放疗，大多数患者可实现完全缓解。早期诊断将导致采用侵袭性较小的治疗方法，从而降低长期晚期并发症的风险。
