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早幼粒细胞性淋巴细胞白血病伴明显母细胞性浆细胞样树突状细胞增生,酷似母细胞性浆细胞样树突状细胞肿瘤:1 例罕见病例报告及文献复习。

Early T-cell precursor lymphoblastic leukemia accompanied by prominent blastic plasmacytoid dendritic cell proliferation mimicking blastic plasmacytoid dendritic cell neoplasm: an exceptional case report and literature review.

机构信息

Department of Laboratory Medicine, West China Hospital, Sichuan University, No 37, Guoxue Xiang, Wuhou District, Chengdu, 610041, Sichuan, China.

Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.

出版信息

J Cancer Res Clin Oncol. 2022 Oct;148(10):2911-2919. doi: 10.1007/s00432-022-04238-0. Epub 2022 Aug 6.

Abstract

PURPOSE

Plasmacytoid dendritic cells (pDCs) are commonly associated with myeloid malignancies. The association between lymphoblastic leukemia and pDCs has been little explored.

CASE PRESENTATION

Here, we report a novel case of early T-cell precursor lymphoblastic leukemia (ETP-ALL) accompanied by prominent proliferation of blastic pDCs mimicking BPDCN. The diagnosis was established based on a comprehensive analysis of morphology, immunophenotype and clinical implications. We also present a literature review and discussion on the differential expression of reactive and neoplastic pDCs, the functional role of pDCs in lymphoblastic leukemia, and the etiological association of normal pDCs and BPDCN.

CONCLUSIONS

The current case demonstrates for the first time that prominent pDC proliferation can be associated with lymphoid neoplasms and can exhibit blastic morphology and immunophenotype. The underlying mechanism of the coexistence of these two blastic populations remains unknown. Further genetic profiling may be required to denote the progressive development of tumor stem cells to the lymphoid, myeloid or dendritic cell lineage. Moreover, the prognostic value of pDCs in hematological neoplasms needs further investigation.

摘要

目的

浆细胞样树突状细胞(pDCs)通常与髓系恶性肿瘤相关。淋系白血病与 pDCs 之间的关联尚未得到充分探索。

病例介绍

本研究报道了一例早期 T 细胞前体急性淋巴细胞白血病(ETP-ALL)病例,其特征为类似于 BPDCN 的成簇性 blast pDC 增殖。诊断基于对形态学、免疫表型和临床意义的综合分析。本研究还对反应性和肿瘤性 pDCs 的差异表达、pDCs 在淋系白血病中的功能作用以及正常 pDCs 和 BPDCN 的病因学关联进行了文献复习和讨论。

结论

本病例首次表明,pDC 大量增殖可与淋巴肿瘤相关,且可表现出成簇性 blast 形态和免疫表型。这两种成簇性细胞群共存的潜在机制尚不清楚。可能需要进一步的基因分析来表示肿瘤干细胞向淋系、髓系或树突状细胞谱系的渐进性发展。此外,pDCs 在血液系统恶性肿瘤中的预后价值需要进一步研究。

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