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伴肝脾T细胞淋巴瘤的T大颗粒淋巴细胞白血病?一例罕见的同时存在肿瘤性T细胞克隆的病例,通过流式细胞术及文献复习揭示

T-Large Granular Lymphocytic Leukemia with Hepatosplenic T-Cell Lymphoma? A Rare Case of Simultaneous Neoplastic T-Cell Clones Highlighted by Flow Cytometry and Review of Literature.

作者信息

Libonati Rossana, Soda Michela, Statuto Teodora, Valvano Luciana, D'Auria Fiorella, D'Arena Giovanni, Pietrantuono Giuseppe, Villani Oreste, Mansueto Giovanna Rosaria, D'Agostino Simona, Di Somma Massimo Dante, Telesca Alessia, Vilella Rocchina

机构信息

Laboratory of Clinical Research and Advanced Diagnostics, Centro di Riferimento Oncologico della Basilicata (IRCCS-CROB), 85028 Rionero In Vulture, Italy.

Laboratory of Clinical Pathology, Centro di Riferimento Oncologico della Basilicata (IRCCS-CROB), 85028 Rionero In Vulture, Italy.

出版信息

Biomedicines. 2024 Apr 30;12(5):993. doi: 10.3390/biomedicines12050993.

DOI:10.3390/biomedicines12050993
PMID:38790955
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11117647/
Abstract

Lymphoproliferative diseases are a heterogeneous set of malignant clonal proliferations of lymphocytes. Despite well-established diagnostic criteria, the diagnosis remains difficult due to their variety in clinical presentation and immunophenotypic profile. Lymphoid T-cell disorders are less common than B-cell entities, and the lack of a clear immunophenotypic characteristic makes their identification hard. Flow cytometry turned out to be a useful tool in diagnosing T-cell disorders and to resolve complicated cases, especially if the number of analyzable neoplastic cells is small. We present a case of a 55-year-old man with simultaneous lymphoproliferative neoplastic T-cell clones, one αβ and the other γδ, identified and characterized by flow cytometry (FC), exploiting the variable expression intensity of specific markers. However, the patient's rapid decline made it impossible to define a differential diagnosis in order to confirm the identity of the γδ clone, which remains uncertain. This case is added to the few other cases already documented in the literature, characterized by the co-existence of T-large granular lymphocytic leukemia (T-LGLL)-αβ and T-LGLL-γδ/Hepatosplenic T-cell lymphoma (HSTCL). Our case underlines the key role of sensitive diagnostic tools in the assessment of potential relationship between the diagnosis, prognosis, and treatment in the two pathologies.

摘要

淋巴增殖性疾病是淋巴细胞的一组异质性恶性克隆增殖性疾病。尽管有既定的诊断标准,但由于其临床表现和免疫表型特征的多样性,诊断仍然困难。淋巴样T细胞疾病比B细胞实体少见,并且缺乏明确的免疫表型特征使得其识别困难。流式细胞术已被证明是诊断T细胞疾病和解决复杂病例的有用工具,特别是在可分析的肿瘤细胞数量较少的情况下。我们报告一例55岁男性同时存在淋巴增殖性肿瘤性T细胞克隆,一个为αβ型,另一个为γδ型,通过流式细胞术(FC)利用特定标志物的可变表达强度进行识别和表征。然而,患者的快速病情恶化使得无法进行鉴别诊断以确认γδ克隆的身份,其身份仍然不确定。该病例被添加到文献中已记录的其他少数病例中,其特征为T大颗粒淋巴细胞白血病(T-LGLL)-αβ和T-LGLL-γδ/肝脾T细胞淋巴瘤(HSTCL)共存。我们的病例强调了敏感诊断工具在评估这两种病理中诊断、预后和治疗之间潜在关系方面的关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b26a/11117647/ae1960732efe/biomedicines-12-00993-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b26a/11117647/d9570402fc79/biomedicines-12-00993-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b26a/11117647/ae1960732efe/biomedicines-12-00993-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b26a/11117647/d9570402fc79/biomedicines-12-00993-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b26a/11117647/ae1960732efe/biomedicines-12-00993-g002.jpg

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本文引用的文献

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Large granular lymphocyte leukemia: An indolent clonal proliferative disease associated with an array of various immunologic disorders.大颗粒淋巴细胞白血病:一种惰性克隆性增殖性疾病,与多种免疫性疾病有关。
Rev Med Interne. 2023 Jun;44(6):295-306. doi: 10.1016/j.revmed.2023.03.014. Epub 2023 Apr 21.
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T-large granular lymphocytic leukemia.T大颗粒淋巴细胞白血病
Blood Res. 2023 Apr 30;58(S1):S52-S57. doi: 10.5045/br.2023.2023037. Epub 2023 Apr 7.
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Not all LGL leukemias are created equal.并非所有的 LGL 白血病都是一样的。
Blood Rev. 2023 Jul;60:101058. doi: 10.1016/j.blre.2023.101058. Epub 2023 Feb 20.
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Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia.α/β 型 T 细胞大颗粒淋巴细胞白血病的分子特征及诊断挑战。
Int J Mol Sci. 2022 Nov 2;23(21):13392. doi: 10.3390/ijms232113392.
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Hepatosplenic T Cell Lymphoma: Diagnostic Conundrum.肝脾T细胞淋巴瘤:诊断难题
Int J Hematol Oncol Stem Cell Res. 2022 Jan 1;16(1):66-73. doi: 10.18502/ijhoscr.v16i1.8444.
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The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms.《世界卫生组织造血与淋巴组织肿瘤分类》第五版:淋巴肿瘤。
Leukemia. 2022 Jul;36(7):1720-1748. doi: 10.1038/s41375-022-01620-2. Epub 2022 Jun 22.
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The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee.成熟淋巴细胞肿瘤国际共识分类:临床咨询委员会报告。
Blood. 2022 Sep 15;140(11):1229-1253. doi: 10.1182/blood.2022015851.
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Large granular lymphocytic leukemia: a brief review.大颗粒淋巴细胞白血病:简要综述。
Am J Blood Res. 2022 Feb 15;12(1):17-32. eCollection 2022.
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T-Cell Large Granular Lymphocyte Leukemia: An Interdisciplinary Issue?T细胞大颗粒淋巴细胞白血病:一个跨学科问题?
Front Oncol. 2022 Feb 10;12:805449. doi: 10.3389/fonc.2022.805449. eCollection 2022.
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Case Report: Large Granular Lymphocyte Leukemia (LGLL)-A Case Series of Challenging Presentations.病例报告:大颗粒淋巴细胞白血病(LGLL)——一系列具有挑战性表现的病例
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