儿童朗格罕斯细胞组织细胞增生症34例临床分析
发布时间：2018-07-30 08:43
【摘要】：目的探讨儿童朗格罕斯细胞组织细胞增生症(LCH)的临床特点和预后,以期提高LCH诊疗水平。方法对34例初发LCH儿童患者进行回顾性分析。结果 34例患者中位年龄14.5个月(22 d至60个月),其中0~2岁的23例、2岁的11例;高危组17例,低危组17例。30例患者接受化疗,6周化疗总有效率67%(20/30),12个月总有效率87%(26/30),3年总生存(OS)率为86%±6%,3年无事件生存(EFS)率为64%±9%。高危组患者6周化疗有效率46.7%,3年OS为72%±12%,3年EFS为46%±13%,均低于低危组(86.7%、100%、82%±9%),差异均有统计学意义(P0.05)。高危组12个月化疗有效率(80%)与低危组(93%)的差异无统计学意义(P0.05);复发率和死亡率均为27%,而低危组无复发和死亡。结论 LCH总体生存率较高,但高危组6周化疗有效率低,远期预后较差。
[Abstract]:Objective to investigate the clinical features and prognosis of Langerhans cell histiocytosis (LCH) in children in order to improve the diagnosis and treatment of LCH. Methods 34 children with primary LCH were retrospectively analyzed. Results the median age of 34 patients was 14.5 months (22 days to 60 months), of which 23 cases were from 0 to 2 years old, 11 cases from 2 years old, and 17 cases from high risk group. The total effective rate of chemotherapy was 67% (20 / 30), 87% (26 / 30) in 12 months, 86% 卤6% in 3-year survival (OS) and 64% 卤9% in event-free (EFS). The effective rate of chemotherapy in high risk group was 46.7%, OS was 72% 卤12 in 3 years, EFS was 46% 卤13 in 3 years, which was lower than that in low risk group (86.7% 卤9%), the difference was statistically significant (P0.05). The effective rate of 12 months chemotherapy in high risk group (80%) was not significantly different from that in low risk group (93%) (P0.05); the recurrence rate and mortality were both 27%, but there was no recurrence and death in low risk group. Conclusion the overall survival rate of LCH is high, but the 6 weeks chemotherapy efficiency is low and the long-term prognosis is poor in high risk group.
【作者单位】： 成都市妇女儿童中心医院小儿血液与肿瘤科;
【分类号】：R725.9
，
本文编号：2154450
[Abstract]:Objective to investigate the clinical features and prognosis of Langerhans cell histiocytosis (LCH) in children in order to improve the diagnosis and treatment of LCH. Methods 34 children with primary LCH were retrospectively analyzed. Results the median age of 34 patients was 14.5 months (22 days to 60 months), of which 23 cases were from 0 to 2 years old, 11 cases from 2 years old, and 17 cases from high risk group. The total effective rate of chemotherapy was 67% (20 / 30), 87% (26 / 30) in 12 months, 86% 卤6% in 3-year survival (OS) and 64% 卤9% in event-free (EFS). The effective rate of chemotherapy in high risk group was 46.7%, OS was 72% 卤12 in 3 years, EFS was 46% 卤13 in 3 years, which was lower than that in low risk group (86.7% 卤9%), the difference was statistically significant (P0.05). The effective rate of 12 months chemotherapy in high risk group (80%) was not significantly different from that in low risk group (93%) (P0.05); the recurrence rate and mortality were both 27%, but there was no recurrence and death in low risk group. Conclusion the overall survival rate of LCH is high, but the 6 weeks chemotherapy efficiency is low and the long-term prognosis is poor in high risk group.
【作者单位】： 成都市妇女儿童中心医院小儿血液与肿瘤科;
【分类号】：R725.9
，
本文编号：2154450
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