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异常的髓系前体细胞 CD13/HLA-DR 表达模式可预测意义未明的克隆性血细胞减少症患者发展为髓系肿瘤。

Abnormal CD13/HLA-DR Expression Pattern on Myeloblasts Predicts Development of Myeloid Neoplasia in Patients With Clonal Cytopenia of Undetermined Significance.

机构信息

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA.

Division of Hematology, Mayo Clinic, Rochester, MN, USA.

出版信息

Am J Clin Pathol. 2022 Oct 6;158(4):530-536. doi: 10.1093/ajcp/aqac083.

Abstract

OBJECTIVES

Patients with clonal cytopenia of undetermined significance (CCUS) are at increased risk of developing myeloid neoplasia (MN). We evaluated whether a simple flow cytometry immunophenotyping (FCIP) assay could differentiate the risk of development of MN in patients with CCUS.

METHODS

Bone marrow aspirates were assessed by FCIP panel in a cohort of 80 patients identified as having CCUS based on next-generation sequencing or cytogenetics from March 2015 to May 2020, with available samples. Flow cytometric assay included CD13/HLA-DR expression pattern on CD34-positive myeloblasts; CD13/CD16 pattern on maturing granulocytic precursors; and aberrant expression of CD2, CD7, or CD56 on CD34-positive myeloblasts. Relevant demographic, comorbidity, and clinical and laboratory data, including the type and extent of genetic abnormalities, were extracted from the electronic health record.

RESULTS

In total, 17 (21%) patients with CCUS developed MN over the follow-up period (median survival follow-up, 28 months [95% confidence interval, 19-31]). Flow cytometry immunophenotyping abnormalities, including the aberrant pattern of CD13/HLA-DR expression, as detected at the time of the diagnosis of CCUS, were significantly associated with risk of developing MN (hazard ratio, 2.97; P = .006). Additional FCIP parameters associated with the development of MN included abnormal expression of CD7 on myeloblasts and the presence vs absence of any FCIP abnormality.

CONCLUSIONS

A simple FCIP approach that includes assessment of CD13/HLA-DR pattern on CD34-positive myeloblasts can be useful in identifying patients with CCUS at higher risk of developing MN.

摘要

目的

具有未确定意义的克隆性血细胞减少症(CCUS)的患者发生髓系肿瘤(MN)的风险增加。我们评估了一种简单的流式细胞免疫表型(FCIP)检测是否可以区分 CCUS 患者发生 MN 的风险。

方法

根据 2015 年 3 月至 2020 年 5 月基于下一代测序或细胞遗传学确定的 CCUS 患者队列,对 80 例患者的骨髓抽吸物进行 FCIP 检测,这些患者有可用样本。流式细胞术检测包括 CD34 阳性原始细胞上 CD13/HLA-DR 表达模式;成熟粒细胞前体上 CD13/CD16 模式;CD34 阳性原始细胞上 CD2、CD7 或 CD56 的异常表达。从电子病历中提取相关的人口统计学、合并症和临床及实验室数据,包括遗传异常的类型和程度。

结果

在随访期间,共有 17 例(21%)CCUS 患者发生 MN(中位随访生存时间为 28 个月[95%置信区间,19-31])。在诊断 CCUS 时检测到的 FCIP 异常,包括 CD13/HLA-DR 表达异常模式,与发生 MN 的风险显著相关(风险比,2.97;P =.006)。与 MN 发生相关的其他 FCIP 参数包括原始细胞上 CD7 的异常表达以及 FCIP 异常的存在与否。

结论

一种简单的 FCIP 方法,包括评估 CD34 阳性原始细胞上 CD13/HLA-DR 模式,可用于识别发生 MN 风险较高的 CCUS 患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46f0/9535519/a8172eca50e0/aqac083f0001.jpg

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