Medfuture Research Center for Translational Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Louis Pasteur Street, Cluj-Napoca, Romania.
Department of Hematology, Ion Chiricuta Clinical Cancer Center, Cluj-Napoca, Romania.
Mol Diagn Ther. 2023 Sep;27(5):593-599. doi: 10.1007/s40291-023-00658-x. Epub 2023 Jun 9.
Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder for which diagnosis is typically straightforward, based on bone marrow morphology and flow cytometry (FC) or immunohistochemistry. Nevertheless, variants present atypical expressions of cell surface markers, as is the case of CD5, for which the differential diagnosis can be more difficult. The aim of the current paper was to describe diagnosis of HCL with atypical CD5 expression, with an emphasis on FC.
The detailed diagnostic methodology for HCL with atypical CD5 expression is presented, including differential diagnosis from other lymphoproliferative diseases with similar pathologic features, by FC analysis of the bone marrow aspirate.
Diagnosis of HCL by means of FC started by gating all events based on side scatter (SSC) versus CD45 and B lymphocytes were selected from the lymphocytes gate as CD45/CD19 positive. The gated cells were positive for CD25, CD11c, CD20, and CD103, while CD10 proved to be dim to negative. Moreover, cells positive for CD3, CD4, and CD8, the three pan-T markers, as well as CD19, showed a bright expression of CD5. The atypical CD5 expression is usually correlated with a negative prognosis and thus chemotherapy with cladribine should be initiated.
HCL is an indolent chronic lymphoproliferative disorder and diagnosis is usually straightforward. However, atypical expression of CD5 renders its differential diagnosis more difficult, but FC is a useful tool that allows an optimal classification of the disease and allows initiation of timely satisfactory therapy.
毛细胞白血病(HCL)是一种慢性淋巴增殖性疾病,其诊断通常基于骨髓形态学和流式细胞术(FC)或免疫组织化学。然而,变体表现出细胞表面标志物的非典型表达,例如 CD5,其鉴别诊断可能更加困难。本文旨在描述具有非典型 CD5 表达的 HCL 的诊断,重点是 FC。
介绍了具有非典型 CD5 表达的 HCL 的详细诊断方法,包括通过 FC 分析骨髓抽吸物对具有相似病理特征的其他淋巴增殖性疾病进行鉴别诊断。
通过 FC 诊断 HCL 首先基于侧向散射(SSC)与 CD45 的所有事件进行门控,并且从淋巴细胞门选择 CD45/CD19 阳性的 B 淋巴细胞。门控细胞对 CD25、CD11c、CD20 和 CD103 呈阳性,而 CD10 呈阴性至弱阳性。此外,对 CD3、CD4 和 CD8(三种泛 T 标志物)以及 CD19 呈阳性的细胞表现出 CD5 的明亮表达。非典型 CD5 表达通常与不良预后相关,因此应开始用 cladribine 进行化疗。
HCL 是一种惰性慢性淋巴增殖性疾病,诊断通常很简单。然而,CD5 的非典型表达使其鉴别诊断更加困难,但 FC 是一种有用的工具,可实现疾病的最佳分类,并可及时启动令人满意的治疗。