Bansal Shashi, Gilhotra Simran, Sharma Upendra, Purohit Abhishek
Department of Pathology-Haematopathology, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, Rajasthan, India.
Department of Pathology, Bhagwan Mahaveer Cancer Hospital and Research Centre, Jaipur, Rajasthan, India.
Indian J Cancer. 2025 Jan 1;62(1):156-161. doi: 10.4103/ijc.ijc_505_21. Epub 2025 May 16.
T-cell acute lymphoblastic leukemia/lymphoma (T-ALL/LBL) is a neoplasm of lymphoblasts that is usually TdT-positive and variably expresses CD1a, CD2, cytoplasmic CD3 (cytoCD3), CD4, CD5, CD7, and CD8. Acute undifferentiated leukemia (AUL) is a rare entity and is defined by the expression of not more than one membrane marker for any given lineage. Blasts express HLA-DR, CD34, and/or CD38 and may be positive for terminal deoxynucleotidyl transferase (TdT). Mixed-phenotype acute leukemia (MPAL) is a hematologic malignancy with immunophenotypic co-expression of markers from at least two cell lineages. In this case series of acute leukemia, we emphasize on key diagnostic or biological points of acute leukemia of ambiguous lineage versus T-ALL and provide a relevant literature review. A diagnosis of AUL can be debatable for our cases; however, the positivity of CD5 and CD7 (bright) ruled out AUL. Although cyCD3 is a T-cell lineage-specific marker of T-ALL, its negativity does not always rule out the diagnosis as several cases have been reported with a negative cyCD3, as reported in our cases.
T细胞急性淋巴细胞白血病/淋巴瘤(T-ALL/LBL)是一种淋巴母细胞肿瘤,通常末端脱氧核苷酸转移酶(TdT)呈阳性,并可变地表达CD1a、CD2、胞质CD3(cyCD3)、CD4、CD5、CD7和CD8。急性未分化白血病(AUL)是一种罕见的疾病,其定义为对于任何给定谱系,表达不超过一种膜标志物。原始细胞表达HLA-DR、CD34和/或CD38,并且可能末端脱氧核苷酸转移酶(TdT)呈阳性。混合表型急性白血病(MPAL)是一种血液系统恶性肿瘤,具有至少两个细胞谱系标志物的免疫表型共表达。在这个急性白血病病例系列中,我们强调了谱系不明确的急性白血病与T-ALL的关键诊断或生物学要点,并提供了相关文献综述。对于我们的病例,AUL的诊断可能存在争议;然而,CD5和CD7(强阳性)阳性排除了AUL。尽管cyCD3是T-ALL的T细胞谱系特异性标志物,但其阴性并不总是排除诊断,正如我们的病例所报道的,已有数例cyCD3阴性的病例报道。