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非洲裔个体中良性遗传性中性粒细胞减少症的自然史。

Natural history of benign ethnic neutropenia in individuals of African ancestry.

机构信息

Hematology Branch, NHLBI, NIH, United States of America.

Department of Hematology and Oncology, Veterans Administration Hospital, Washington DC, United States of America.

出版信息

Blood Cells Mol Dis. 2019 Jul;77:12-16. doi: 10.1016/j.bcmd.2019.01.009. Epub 2019 Mar 15.

Abstract

BACKGROUND

Benign ethnic neutropenia (BEN), defined by neutrophil count <1.5 k/μL in the absence of other causes, is an asymptomatic condition more commonly observed in individuals of African ancestry. However, the natural history of this condition has been less well described.

METHODS

Individuals with BEN were retrospectively identified by chart review or referral to hematology clinics. They were then invited to enroll in a prospective natural history study. Retrospective and prospective clinical and laboratory data were combined for descriptive analyses.

FINDINGS

46 participants, younger and older adults from 2 institutions, had BEN. Hypertension was reported in 30%, musculoskeletal disorders in 15%, and upper respiratory infection in 33% of these adults. Their leukopenia resulted from isolated neutropenia, ranging from 1000 and 1500 cells/μL. The severity of infections was mild and the frequency was similar to other healthy individuals in the ambulatory clinic.

INTERPRETATION

In this group of BEN participants, their leukopenia was stable over time, and they had low rates of infections or common medical disorders, confirming the benign nature of this condition. The presence of BEN in children, younger adults, and older adults suggest a hereditary pattern for BEN.

摘要

背景

良性种族中性粒细胞减少症(BEN)定义为中性粒细胞计数<1.5×10^9/L,无其他原因,是一种无症状的情况,更常见于非洲裔个体。然而,这种情况的自然史描述得还不够详细。

方法

通过病历回顾或转介到血液科诊所,回顾性地确定了 BEN 患者。然后邀请他们参加前瞻性自然史研究。将回顾性和前瞻性的临床和实验室数据结合起来进行描述性分析。

结果

2 家机构的 46 名年龄较大和较小的参与者患有 BEN。这些成年人中 30%有高血压,15%有肌肉骨骼疾病,33%有上呼吸道感染。他们的白细胞减少症是由孤立性中性粒细胞减少引起的,范围在 1000 到 1500 个细胞/μL。感染的严重程度较轻,频率与门诊的其他健康个体相似。

解释

在这组 BEN 参与者中,他们的白细胞减少症随时间稳定,感染或常见疾病的发生率较低,证实了这种情况的良性性质。BEN 存在于儿童、年轻成年人和老年人中,提示 BEN 具有遗传性。

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