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埃及慢性淋巴细胞白血病患者队列中自身免疫性血细胞减少症的患病率及预后意义

The prevalence and prognostic significance of autoimmune cytopenias in a cohort of Egyptian patients with chronic lymphocytic leukemia.

作者信息

Atef Basma, Azmy Emad, Aladle Doaa, Mabed Mohamed

机构信息

The Hematology Unit, Oncology Center, Mansoura University, Mansoura, Egypt.

The Clinical Pathology Department, Faculty of Medicine, Mansoura University, Mansoura, Egypt.

出版信息

Hematol Oncol Stem Cell Ther. 2019 Jun;12(2):97-104. doi: 10.1016/j.hemonc.2019.01.004. Epub 2019 Feb 20.

Abstract

OBJECTIVE/BACKGROUND: The impact of autoimmune cytopenias (AICs) on the chronic lymphocytic leukemia (CLL) clinical course and its prognostic significance remain a matter of controversial debate. This could be due to exclusion of patients with cytopenia from most clinical trials for this particular complication and the lack of standard diagnostic criteria and treatment approaches. We herein evaluate the prevalence and the prognostic significance of AICs among patients with CLL.

METHODS

This is an observational retrospective study. Data on 101 patients with CLL were derived from the Oncology Center, Mansoura University, Egypt, database, which contains information on demographic and clinical characteristics at diagnosis and follow-up records.

RESULTS

The prevalence of immune cytopenias was 11.9% among patients studied. Autoimmune hemolytic anemia was the most common autoimmune form in patients with cytopenia due to pure immune etiology (C immune group) with a prevalence of 6.9%. Patients with AICs and those in the C immune subgroup presented with more unfavorable parameters. Besides, patients with AICs showed lesser response to treatment and on restaging after initial treatment, significantly more patients without AICs moved to a more favorable stage. However, no parallel significant difference in the overall survival was found between patients without AICs and those with AICs or with immune and combined or infiltrative cytopenia.

CONCLUSION

We have shown a prevalence of 11.8% for AIC among our CLL patients. AIC was associated with unsatisfactory normalization of the hematological parameters even with therapy and lower number of patients with CLL downstaging in comparison with patients without AIC. These results suggest that AIC is a fingerprint of a biologically more aggressive disease even if no significant impact on overall survival was found.

摘要

目的/背景:自身免疫性血细胞减少症(AIC)对慢性淋巴细胞白血病(CLL)临床病程的影响及其预后意义仍存在争议。这可能是由于大多数针对这一特殊并发症的临床试验排除了血细胞减少症患者,且缺乏标准的诊断标准和治疗方法。我们在此评估CLL患者中AIC的患病率及其预后意义。

方法

这是一项观察性回顾性研究。101例CLL患者的数据来自埃及曼苏拉大学肿瘤中心数据库,该数据库包含诊断时的人口统计学和临床特征信息以及随访记录。

结果

在所研究的患者中,免疫性血细胞减少症的患病率为11.9%。自身免疫性溶血性贫血是纯免疫病因导致血细胞减少症患者(C免疫组)中最常见的自身免疫形式,患病率为6.9%。AIC患者和C免疫亚组患者的参数更不理想。此外,AIC患者对治疗的反应较小,初始治疗后重新分期时,无AIC的患者明显更多地进入更有利的阶段。然而,无AIC患者与有AIC患者或有免疫性、合并性或浸润性血细胞减少症患者之间在总生存期方面未发现平行的显著差异。

结论

我们的CLL患者中AIC的患病率为11.8%。AIC与血液学参数即使经过治疗也未能令人满意地恢复正常有关,与无AIC的患者相比,CLL病情缓解的患者数量较少。这些结果表明,AIC是一种生物学上更具侵袭性疾病的特征,即使未发现对总生存期有显著影响。

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