Department of Medicine, University of Massachusetts Medical School, Baystate Medical Center, Springfield, MA 01103, USA.
Department of Medicine, University of Massachusetts Medical School, Baystate Medical Center, Springfield, MA 01103, USA.
Blood Rev. 2019 Sep;37:100586. doi: 10.1016/j.blre.2019.06.003. Epub 2019 Jun 21.
Benign ethnic neutropenia (BEN) is one of the most common causes of chronic neutropenia seen in individuals of African, Middle Eastern and West Indian descent, affecting many individuals worldwide. Despite its prevalence, many physicians are not familiar with this benign condition, resulting in unnecessary evaluation and testing for neutropenia in otherwise healthy individuals. Clinically, patients with BEN are at no increased risk of infection despite their neutropenia. Implications of this condition are highlighted in those patients receiving therapies that have a known side effect of neutropenia, most commonly chemotherapy agents. Studies have suggested that disparities in survival among those patients receiving chemotherapy between patients of European decent and African decent may be attributed to measured neutropenia in these populations, questioning whether BEN could be an influential factor. This review encompasses all aspects of benign ethnic neutropenia, providing information about this condition and helping to guide clinical decision-making as to when an aggressive work up and referral are indicated and when it is appropriate to monitor. Additionally, we review the role of genetic studies in identifying the genes related to BEN, summarize the theories that offer the most accepted mechanisms behind the condition, and address the importance of pursuing larger studies to assess the implication of BEN in oncology patients as well as patients taking neutropenia-causing medications.
良性种族中性粒细胞减少症(BEN)是非洲、中东和西印度裔个体中最常见的慢性中性粒细胞减少症原因之一,影响着全世界许多人。尽管这种情况很常见,但许多医生并不熟悉这种良性疾病,导致对其他方面健康的个体进行不必要的中性粒细胞减少症评估和检查。临床上,尽管存在中性粒细胞减少症,但患有 BEN 的患者没有增加感染的风险。在接受已知具有中性粒细胞减少副作用的治疗的患者中,这种情况的影响更为明显,最常见的是化疗药物。研究表明,接受化疗的欧洲裔和非洲裔患者之间的生存率差异可能归因于这些人群中中性粒细胞减少症的测量,这引发了一个问题,即 BEN 是否可能是一个有影响的因素。本综述涵盖了良性种族中性粒细胞减少症的所有方面,提供了有关该疾病的信息,并有助于指导临床决策,确定何时需要进行积极的检查和转介,以及何时需要监测。此外,我们还回顾了遗传研究在确定与 BEN 相关的基因方面的作用,总结了最能解释该疾病背后机制的理论,并探讨了进行更大规模研究的重要性,以评估 BEN 在肿瘤患者以及服用导致中性粒细胞减少症药物的患者中的意义。