Weinberg Olga K, Bueso-Ramos Carlos E, Kanagal-Shamanna Rashmi
Department of Hematopathology, The University of Texas Southwestern Medical Center, Dallas, TX, United States.
Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, United States.
Am J Clin Pathol. 2025 Jul 11;164(1):26-35. doi: 10.1093/ajcp/aqaf015.
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, γ/δ 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.
2023年血液病理学学会/欧洲血液病理学协会研讨会探讨了T细胞和自然杀伤（NK）细胞淋巴瘤/白血病诊断与分类的进展。
研讨会第8场会议收集了38例不同类型的未成熟T细胞和NK细胞淋巴瘤/白血病病例，以及谱系不明的急性白血病（ALAL）和其他杂项病例，包括惰性T淋巴细胞增殖病例。
20例T淋巴细胞白血病/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，中位年龄为21.5岁。男性占主导（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%）常有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病例。
本次会议强调了具有细胞毒性标志物表达、γ/δ表型、更成熟免疫表型的不寻常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，1例患者发展为成熟NK细胞表型。ALAL患者出现在先前治疗或种系突变的不寻常情况下。
