Klimza Małgorzata J, Sońta-Jakimczyk Danuta J
Studium Doktoranckiego Wydziału Lekarskiego w Zabrzu.
Wiad Lek. 2005;58(11-12):622-5.
Leukemias are the most common malignant diseases in childhood, with acute lymphoblastic leukemia (ALL) being the most frequent subtype. Diagnosis and treatment of ALL remains an important issue in pediatric practice. The great variety of prognostic factors has been recognized and applied for the stratification into different risk groups and consequently for selection of the most appropriate treatment. The most significant prognostic factors include age, sex, white blood count at diagnosis, infiltration of the extramedullary organs, and central nervous system involvement. The experience of numerous pediatric hemato-oncology centers has shown that above-mentioned prognostic criteria are not sufficient and they emphasize the need for determination of the response to glucocorticosteroids at day 8 of treatment. A retrospective analysis of treatment results in childhood in ALL patients with regard to steroid response has been performed. The study aimed at analysis whether there is any association between the long-term event-free survival (EFS) in patients with good and poor response to prednisone. The study group consisted of 179 children, aged 2 to 17 years, qualified into low and medium risk groups, treated according to the BFM-86 and BFM-90 protocols in the Department of Pediatrics and Hematology of Silesian Center of Pediatrics in Zabrze between 1986 and 1996. In the study group, 89.9% (161) of children showed good prednisone response (PGR) as compared to 10.1% of children with poor response to steroids (PPR). Cumulative probability of 15-year EFS for the PGR group was 70% and significantly differed from EFS in PPR group (39%; p = 0.006). In the study group, the children showing good response to the initial treatment with prednisone have higher chance for durable remission and subsequent cure. In conclusion, response to glucocorticosteroids employed in the initial treatment of children with ALL, should be included in the stratification into prognostic groups.
白血病是儿童时期最常见的恶性疾病，急性淋巴细胞白血病（ALL）是最常见的亚型。ALL的诊断和治疗仍是儿科临床实践中的一个重要问题。人们已经认识到多种预后因素，并将其应用于不同风险组的分层，从而选择最合适的治疗方法。最重要的预后因素包括年龄、性别、诊断时的白细胞计数、髓外器官浸润和中枢神经系统受累情况。众多儿科血液肿瘤中心的经验表明，上述预后标准并不充分，他们强调需要在治疗第8天确定对糖皮质激素的反应。对ALL患儿治疗结果进行了关于类固醇反应的回顾性分析。该研究旨在分析对泼尼松反应良好和反应不佳的患者的长期无事件生存（EFS）之间是否存在关联。研究组由179名年龄在2至17岁之间、符合低危和中危组标准的儿童组成，他们于1986年至1996年期间在扎布热西里西亚儿科中心儿科和血液科按照BFM - 86和BFM - 90方案进行治疗。在研究组中，89.9%（161名）儿童对泼尼松反应良好（PGR），而对类固醇反应不佳（PPR）的儿童占10.1%。PGR组15年EFS的累积概率为70%，与PPR组的EFS（39%；p = 0.006）有显著差异。在研究组中，对初始泼尼松治疗反应良好的儿童有更高的持久缓解和后续治愈机会。总之，ALL患儿初始治疗中对糖皮质激素的反应应纳入预后分组的分层中。
