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慢性髓性白血病患者临床血液学特征、分子谱及对伊马替尼反应的比较研究:儿童和青少年与成人的对比

Comparative Study of Clinico-hematological Features, Molecular Spectrum and Response to Imatinib in Chronic Myelogenous Leukemia Patients: Pediatric and Adolescent Versus Adults.

作者信息

Tahlan Anita, Varma Neelam, Naseem Shano, Bansal Deepak, Binota Jogeshwar, Sood Anil, Sachdeva Man Updesh Singh, Malhotra Pankaj, Varma Subhash

机构信息

1Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

2Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Indian J Hematol Blood Transfus. 2018 Jan;34(1):19-24. doi: 10.1007/s12288-017-0827-7. Epub 2017 May 9.

Abstract

Chronic myelogenous leukemia (CML) is a rare disease in children, accounting for approximately 3% of leukemias in children and adolescents, with an annual incidence of 1 case per million children in western countries. This study was conducted, at PGIMER, Chandigarh. Ninety eight patients, 48 in children and adolescents group, and 50 in adult group were included in the study. Their hematological profiles along with the bone marrow findings were analyzed. The diagnosis of CML was confirmed by cytogenetics and/or molecular analysis. The complete hematological response (CHR) was analyzed at 3 months and cytogentic response (CgR) at 12 months after starting imatinib therapy. Compared to adults, pediatric and adolescent patients were more symptomatic at presentation (93.5 vs. 75%). Among symptomatic patients, massive splenomegaly (>10 cm), higher total leucocyte and platelet counts were seen more frequently in pediatric patients. The most common transcript in both groups was e14a2. The distribution of pediatric and adolescent cases in Sokal, Hasford and EUTOS score, showed only statistically significant difference for low risk Sokal group, which had more patients in pediatric group. Compared to adults, pediatric and adolescent patients had similar CHR rate (91.3 vs. 92%), but showed lesser major CgR rate (90.9 vs. 95.5%) however, this was not statistically significant.

摘要

慢性粒细胞白血病(CML)在儿童中是一种罕见疾病,约占儿童和青少年白血病的3%,在西方国家每年每百万儿童中的发病率为1例。本研究在昌迪加尔的全印医学科学研究所(PGIMER)进行。该研究纳入了98例患者,其中儿童和青少年组48例,成人组50例。分析了他们的血液学特征以及骨髓检查结果。CML的诊断通过细胞遗传学和/或分子分析得以证实。在开始伊马替尼治疗后3个月分析完全血液学缓解(CHR)情况,12个月时分析细胞遗传学缓解(CgR)情况。与成人相比,儿童和青少年患者在就诊时症状更明显(93.5%对75%)。在有症状的患者中,儿童患者更频繁出现巨大脾肿大(>10厘米)、更高的白细胞总数和血小板计数。两组中最常见的转录本均为e14a2。儿童和青少年病例在索卡尔(Sokal)、哈斯福德(Hasford)和EUTOS评分中的分布情况显示,仅低风险索卡尔组存在统计学显著差异,该组中儿童患者更多。与成人相比,儿童和青少年患者的CHR率相似(91.3%对92%),但主要CgR率较低(90.9%对95.5%),不过这并无统计学显著性差异。

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How I treat childhood CML.我是如何治疗儿童慢性髓性白血病的。
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Chronic myeloid leukaemia.慢性髓细胞白血病
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