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Clinical characteristics and outcome of pediatric patients diagnosed with Langerhans cell histiocytosis in pediatric hematology and oncology centers in Poland.
BMC Cancer ( IF 3.8 ) Pub Date : 2020-09-11 , DOI: 10.1186/s12885-020-07366-3
Anna Raciborska 1 , Katarzyna Bilska 1 , Jadwiga Węcławek-Tompol 2 , Olga Gryniewicz-Kwiatkowska 3 , Małgorzata Hnatko-Kołacz 4 , Joanna Stefanowicz 5 , Anna Pieczonka 6 , Katarzyna Jankowska 7 , Filip Pierelejewski 8 , Tomasz Ociepa 9 , Grażyna Sobol-Milejska 10 , Katarzyna Muszyńska-Rosłan 11 , Olga Michoń 12 , Wanda Badowska 13 , Monika Radwańska 14 , Katarzyna Drabko 15
Affiliation  

Langerhans cell histiocytosis (LCH) affects 1–2 in 1,000,000 people. The disease is not associated with increased risk of treatment failure (especially among older children), but appropriate procedures implemented in advance can eliminate complications which might appear and significantly worsen the patients’ quality of life. Thus, we sought to evaluate the clinical features, management, and outcome of children with LCH treated in Polish pediatric hematology-oncology centers. One hundred eighty two patients with LCH were treated according to the Histiocytic Society Guidelines between 2010 and 2017. The participating centers were requested to provide the following data: demographic, clinical, as well as local or systemic treatment data and patients’ outcome. Overall survival (OS) and event free survival (EFS) were estimated by Kaplan-Meier methods and compared using the log-rank test. Sixty nine percent of children were classified as single system (SS). The patients with SS disease were significantly older as compared to the children with multisystem disease (MS), 6 vs. 2.3 years respectively (p 0.003). Bones were involved in 76% of patients. Systemic treatment was applied to 47% of children with SS disease and 98% with MS disease. Fourteen patients relapsed while two children died. OS and EFS in entire group were 0.99 and 0.91 respectively (with median follow-up 4.3 years). The treatment of LCH in Polish centers was effective, however, new approaches, including mutation analyses and good inter-center cooperation, are needed to identify patients who might require modification or intensification of treatment.

中文翻译:

波兰小儿血液学和肿瘤学中心诊断为Langerhans细胞组织细胞增生症的小儿患者的临床特征和结局。

朗格汉斯细胞组织细胞增生症(LCH)在1,000,000人中影响1-2。该疾病与治疗失败的风险增加无关(尤其是在较大的儿童中),但是提前实施适当的程序可以消除可能出现的并发症,并显着恶化患者的生活质量。因此,我们试图评估在波兰儿科血液肿瘤中心接受治疗的LCH儿童的临床特征,治疗和结局。根据2010年至2017年间的组织细胞学会指南,对182名LCH患者进行了治疗。请参与中心提供以下数据:人口统计学,临床以及局部或全身治疗数据以及患者预后。通过Kaplan-Meier方法估算总生存期(OS)和无事件生存期(EFS),并使用对数秩检验进行比较。69%的儿童被归为单一系统(SS)。与患有多系统疾病(MS)的儿童相比,患有SS疾病的患者年龄要大得多,分别为6岁和2.3岁(P = 0.003)。骨头参与了76%的患者。对47%的SS患儿和98%的MS患儿进行了全身治疗。十四名患者复发,两名儿童死亡。整个组的OS和EFS分别为0.99和0.91(中位随访时间为4.3年)。在波兰中心对LCH的治疗很有效,但是,新方法包括突变分析和良好的中心间合作,
更新日期:2020-09-11
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