發(fā)布時(shí)間：2018-07-30 08:43

【摘要】：目的探討兒童朗格罕斯細(xì)胞組織細(xì)胞增生癥(LCH)的臨床特點(diǎn)和預(yù)后,以期提高LCH診療水平。方法對(duì)34例初發(fā)LCH兒童患者進(jìn)行回顧性分析。結(jié)果 34例患者中位年齡14.5個(gè)月(22 d至60個(gè)月),其中0~2歲的23例、2歲的11例;高危組17例,低危組17例。30例患者接受化療,6周化療總有效率67%(20/30),12個(gè)月總有效率87%(26/30),3年總生存(OS)率為86%±6%,3年無(wú)事件生存(EFS)率為64%±9%。高危組患者6周化療有效率46.7%,3年OS為72%±12%,3年EFS為46%±13%,均低于低危組(86.7%、100%、82%±9%),差異均有統(tǒng)計(jì)學(xué)意義(P0.05)。高危組12個(gè)月化療有效率(80%)與低危組(93%)的差異無(wú)統(tǒng)計(jì)學(xué)意義(P0.05);復(fù)發(fā)率和死亡率均為27%,而低危組無(wú)復(fù)發(fā)和死亡。結(jié)論 LCH總體生存率較高,但高危組6周化療有效率低,遠(yuǎn)期預(yù)后較差。
[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% 鹵6% in 3-year survival (OS) and 64% 鹵9% in event-free (EFS). The effective rate of chemotherapy in high risk group was 46.7%, OS was 72% 鹵12 in 3 years, EFS was 46% 鹵13 in 3 years, which was lower than that in low risk group (86.7% 鹵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.
【作者單位】： 成都市婦女兒童中心醫(yī)院小兒血液與腫瘤科;
【分類(lèi)號(hào)】：R725.9
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本文編號(hào)：2154450