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T 细胞和 NK 细胞淋巴母细胞白血病/淋巴瘤:2023 年 SH/EAHP 研讨会报告

英文原题:T and NK lymphoblastic leukemia/lymphoma: Report from the 2023 SH/EAHP Workshop.

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T and NK lymphoblastic leukemia/lymphoma: Report from the 2023 SH/EAHP Workshop.

PubMed 2025/07/11(内容时间) Am J Clin Pathol Q2 · IF 2.3(JCR 2025)

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研究概要

本次会议重点讨论了表达细胞毒性标志物、/表型、更成熟免疫表型的罕见 T-LBL/L 病例,以及难以分类的 ALAL。在 T-ALL 病例中,发现了有趣的复发变化,其中 1 例患者出现了成熟 NK 细胞表型。ALAL 患者表现为既往治疗或胚系突变这一不寻常的背景。

研究思路结论见上方概要

2023年血液病理学会/欧洲血液病理学协会研讨会讨论了T细胞和自然杀伤(NK)细胞淋巴瘤/白血病诊断和分类的进展。

第8场研讨会收集了38例未成熟T细胞和NK细胞淋巴瘤/白血病、谱系不明急性白血病(ALAL)以及其他杂类病例,包括惰性T淋巴母细胞增殖。

20例T淋巴母细胞白血病/淋巴瘤(T-LBL/L)患者和3例早期T细胞前体急性淋巴细胞白血病(ETP-ALL)患者的中位年龄为21.5岁。男性占优势(占所有病例的70%),40%有纵隔肿块。60%的病例CD34和TdT均为阴性。此外,提交了7例ALAL和3例混合表型急性白血病T/髓系亚型,中位发病年龄为16岁(范围11-56岁),大多数患者(67%)常表现为淋巴结肿大或脾肿大。还提交了1例NK急性淋巴细胞白血病。

展开英文摘要原文

The 2023 Society for Hematopathology/European Association for Hematopathology Workshop addressed advancements in the diagnosis and classification of T- and natural killer (NK)-cell lymphomas/leukemias.

Session 8 of the workshop collected a diverse set of 38 cases of immature T- and NK-cell lymphoma/leukemias, as well as acute leukemia of ambiguous lineage (ALAL) and other miscellaneous cases, including indolent T-lymphoblastic proliferations.

Twenty patients with T-lymphoblastic leukemia/lymphoma (T-LBL/L) and 3 patients with early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) presented at a median age of 21.5 years. Male sex was predominant (70% of all cases), with 40% having a mediastinal mass. Cases (60%) were negative for both CD34 and TdT. In addition, 7 ALAL and 3 mixed phenotype acute leukemia, T/myeloid subtypes were submitted with a median presenting age of 16 (range, 11-56) years, and most patients (67%) frequently showed adenopathy or splenomegaly. A single case of NK acute lymphoblastic leukemia was also submitted.

This session highlighted unusual T-LBL/L cases with expression of cytotoxic markers, / phenotype, a more mature immunophenotype, and ALAL that are challenging to classify. Among T-ALL cases, interesting relapse changes were identified, with 1 patient developing a mature NK-cell phenotype. Patients with ALAL presented in an unusual setting of prior therapy or a germline mutation.

论文信息

作者
Weinberg OK、Bueso-Ramos CE、Kanagal-Shamanna R
第一作者单位
Department of Hematopathology, The University of Texas Southwestern Medical Center, Dallas, TX, United States.United States
通讯作者单位
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States.United States
期刊
American journal of clinical pathology2025 Jul 11
原文标识
PubMed 40646675 · DOI 10.1093/ajcp/aqaf015