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来自巴基斯坦的一组17p缺失的慢性淋巴细胞白血病患者的临床血液学参数及预后

Clinicohematological parameters and outcomes in a cohort of chronic lymphocytic leukemia patients with Deletion 17p from Pakistan.

作者信息

Mahmood Rafia, Khan Saleem Ahmed, Altaf Chaudhry, Malik Hamid Saeed, Khadim Muhammad Tahir

机构信息

Department of Haematology, Armed Forces Institute of Pathology, Rawalpindi, Pakistan.

出版信息

Blood Res. 2018 Dec;53(4):276-280. doi: 10.5045/br.2018.53.4.276. Epub 2018 Dec 17.

DOI:10.5045/br.2018.53.4.276
PMID:30588463
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6300684/
Abstract

BACKGROUND

Chronic lymphocytic leukemia (CLL) exhibits profound heterogeneity in its clinical course. Its clinicohematological and cytogenetic features play a significant role in determining the clinical course and in predicting the treatment response and prognosis. In this context, 17p deletion is known to predict a poor prognosis, as these cases are refractory to conventional therapy. This study aimed to evaluate the clinicohematological characteristics, outcomes, and prognostic factors among CLL patients with and without del 17p in Pakistan.

METHODS

This prospective observational study was conducted at the Department of Haematology, Armed Forces Institute of Pathology (Rawalpindi, Pakistan) between January 2013 and December 2017. Patients were diagnosed based on the International Workshop on Chronic Lymphocytic Leukaemia IWCLL criteria, their clinicohematological parameters were recorded, and cytogenetic analyses were performed. The time from diagnosis to treatment and the 2-year overall survival rate were also evaluated.

RESULTS

We evaluated 130 CLL cases, including 24 patients (18.5%) with del 17p, who included 18 men (75%) and 6 women (25%). The median age was 68 years. Binet stage C was detected at the presentation in 16 patients (67%). Treatment was administered to 14 patients (70%) at a median interval of 11 months (range, 0-28 mo) after diagnosis. The overall response rate was 64.3%, the median event-free survival was 9 months (range, 1-23 mo), and the 2-year overall survival rate was 65%.

CONCLUSION

Del 17p is relatively common in Pakistan, and patients harboring this deletion had poor treatment response and survival outcomes.

摘要

背景

慢性淋巴细胞白血病(CLL)在临床病程中表现出显著的异质性。其临床血液学和细胞遗传学特征在决定临床病程、预测治疗反应和预后方面起着重要作用。在这种情况下,已知17p缺失预示着预后不良,因为这些病例对传统治疗难治。本研究旨在评估巴基斯坦有和没有17p缺失的CLL患者的临床血液学特征、结局和预后因素。

方法

这项前瞻性观察性研究于2013年1月至2017年12月在巴基斯坦拉瓦尔品第武装部队病理研究所血液科进行。患者根据慢性淋巴细胞白血病国际研讨会(IWCLL)标准进行诊断,记录其临床血液学参数,并进行细胞遗传学分析。还评估了从诊断到治疗的时间以及2年总生存率。

结果

我们评估了130例CLL病例,其中24例(18.5%)有17p缺失,包括18名男性(75%)和6名女性(25%)。中位年龄为68岁。16例患者(67%)初诊时为Binet分期C期。14例患者(70%)在诊断后中位间隔11个月(范围0 - 28个月)接受了治疗。总缓解率为64.3%,中位无事件生存期为9个月(范围1 - 23个月),2年总生存率为65%。

结论

17p缺失在巴基斯坦相对常见,携带这种缺失的患者治疗反应和生存结局较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1046/6300684/fd53e142c2ad/br-53-276-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1046/6300684/fd53e142c2ad/br-53-276-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1046/6300684/fd53e142c2ad/br-53-276-g001.jpg

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