儿童和青少年慢性粒细胞白血病:印度西部的一项随访研究。

IF 0.8 4区 医学 Q4 PATHOLOGY Indian Journal of Pathology and Microbiology Pub Date : 2023-07-01 DOI:10.4103/ijpm.ijpm_462_21
Sangita A Vanik, Dhaval Jetly, Biren Parikh, Karthik Dhandapani, Rukmini Bezbaruah
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引用次数: 0

摘要

背景:慢性粒细胞白血病(CML)在儿童和青少年年龄组中相对罕见。本研究的目的是评估儿童和青少年年龄组慢性粒细胞白血病的临床、血液病理学和生化参数,并评估一线酪氨酸激酶抑制剂(TKI)的治疗反应及其与成人预后评分系统的相关性。材料和方法:对我院登记的44例BCR-ABL1阳性儿童和青少年慢性粒细胞白血病病例进行回顾性研究。使用医院软件评估临床和实验室参数。监测治疗反应,并使用数学计算进行评分。结果:平均年龄11.6(±4.7)岁。血红蛋白中位数为8.4 g/dL,63.6%的病例显示白细胞计数>250000/μL。平均随访21个月。在治疗过程中,共有97.7%和78.1%的病例分别获得了完全血液学反应(CHR)和分子反应。Sokal和欧洲治疗和结果研究(EUTOS)评分较低的患者人数最多。75%的病例在3个月时达到CHR,而Sokal和EUTOS评分较低的慢性粒细胞白血病慢性期(CP)病例分别有73.6%和78.6%在3个月中达到CHR。结论:本研究表明,儿童和青少年年龄组的慢性粒细胞白血病患者在诊断时通常存在较高的白细胞计数。预后评分系统与治疗反应的相关性在统计学上不显著。然而,需要进行更大规模的队列研究来确定TKI在儿童和青少年CML中的治疗反应及其与预后评分系统的相关性。
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Pediatric and adolescent chronic myeloid leukemia: A follow-up study in Western India.

Background: Chronic myeloid leukemia (CML) is relatively rare in pediatric and adolescent age groups. The purpose of this study was to evaluate the clinical, hematopathological, and biochemical parameters of CML in pediatric and adolescent age groups, along with an assessment of the treatment response with first-line tyrosine kinase inhibitors (TKI) and its correlation with the prognostic scoring systems of adults.

Materials and methods: A retrospective study of 44 Breakpoint Cluster Region-Abelson leukemia virus (BCR-ABL1)-positive pediatric and adolescent CML cases registered at our hospital was done. The clinical and laboratory parameters were evaluated using hospital software. The treatment response was monitored and scoring was performed using mathematical calculations.

Results: The mean age was 11.6 (±4.7) years. The median hemoglobin was 8.4 g/dL and 63.6% of the cases showed white blood cell (WBC) counts >250,000/μL. The average follow-up was 21 months. A total of 97.7 and 78.1% cases achieved complete hematological response (CHR) and molecular response, respectively, during the treatment course. The maximum number of patients had low Sokal and European treatment and Outcomes Study (EUTOS) scores. Seventy-five per cent of the cases achieved CHR at 3 months, while 73.6 and 78.6% CML-Chronic phase (CP) cases with low Sokal and EUTOS scores achieved CHR at 3 months, respectively.

Conclusion: This study revealed that the CML cases in pediatric and adolescent age groups are normally present with higher WBC counts at the time of diagnosis. The association of the prognostic scoring system with treatment response was statistically insignificant. However, a larger cohort study is needed to determine the treatment response of TKI in children and adolescent CML and its correlation with the prognostic scoring systems.

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CiteScore
1.20
自引率
0.00%
发文量
422
审稿时长
1 months
期刊介绍: The journal will cover studies related to pathology including morbid anatomy, surgical pathology, clinical pathology, diagnostic cytopathology including gynecologic cytology and aspiration cytology, hematology including immuno-hematology and medical microbiology. The journal gives preference to clinically oriented studies over experimental and animal studies. The Journal would publish peer-reviewed original research papers, case reports, systematic reviews, meta-analysis, letters to the editor and brief communications. Review articles on current topics usually are invited by the editor.
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