Gujral S, Polampalli S, Badrinath Y, Kumar A, Subramanian P G, Nair R, Sengar M, Nair C
Department of Pathology, Tata Memorial Hospital, Mumbai, India.
Indian J Cancer. 2010 Apr-Jun;47(2):189-93. doi: 10.4103/0019-509X.63020.
Mature T/NK cell lymphomas (MTNKL) presenting as leukemia are rare and show considerable overlapping of clinical, morphological and immunophenotypic features.
Critical analysis of the morphology and immunophenotypic profile of MTNKL.
We reviewed 380 consecutive cases of mature lymphoid neoplasm that presented as leukemia and were diagnosed on morphology and immunophenotyping of bone marrow and/or peripheral blood samples.
Peripheral blood and bone marrow involvement was seen in all cases. MTNKL constituted 4% (nine cases) of all mature lymphoid neoplasms presenting as leukemia. It included four cases of T-large granular leukemia (T-LGL), two of T-cell prolymphocytic leukemia small cell variant (T-PLL), two of adult T-cell leukemia/lymphoma (ATLL) and one of primary cutaneous gamma delta T-cell lymphoma (PCGDTCL). T-LGL revealed CD4-/CD8+ phenotype in three, and CD4+/CD8+ phenotype in one case. CD56 was absent in all the cases of T-LGL. One case of T- PLL small cell variant showed CD4+/CD8- phenotype, while the other revealed CD4-/CD8+ phenotype. Both cases of ATLL showed CD4+/CD8+/CD25+ phenotype. The single case of PCGDTCL showed CD4-/CD8- phenotype pattern. CD3 and CD5 were expressed in all MTNKL. CD7 was absent in three cases of T-LGL. TCRalpha/beta was performed in three cases of T-LGL and was positive in all. TCRalpha/beta was also seen in both the cases of T-PLL small variant. However, TCRalpha/beta was seen in the single case of PCGDTCL.
Mature nodal T/NK cell neoplasms are rare and MTNKL presenting as leukemia are even rarer. There is an overlap between the immunophenotypic profiles of different MTNKL subtypes and elaborate T/NK cell panels are required for their evaluation.
表现为白血病的成熟T/NK细胞淋巴瘤（MTNKL）较为罕见，在临床、形态学及免疫表型特征方面存在相当程度的重叠。
对MTNKL的形态学及免疫表型特征进行批判性分析。
我们回顾了380例连续的成熟淋巴细胞肿瘤病例，这些病例均表现为白血病，并通过骨髓和/或外周血样本的形态学及免疫表型分析进行诊断。
所有病例均可见外周血和骨髓受累。MTNKL占所有表现为白血病的成熟淋巴细胞肿瘤的4%（9例）。其中包括4例T大颗粒淋巴细胞白血病（T-LGL）、2例T细胞幼淋巴细胞白血病小细胞变异型（T-PLL）、2例成人T细胞白血病/淋巴瘤（ATLL）以及1例原发性皮肤γδT细胞淋巴瘤（PCGDTCL）。3例T-LGL显示CD4-/CD8+表型，1例显示CD4+/CD8+表型。所有T-LGL病例均无CD56表达。1例T-PLL小细胞变异型显示CD4+/CD8-表型，另1例显示CD4-/CD8+表型。2例ATLL均显示CD4+/CD8+/CD25+表型。唯一的PCGDTCL病例显示CD4-/CD8-表型模式。所有MTNKL均表达CD3和CD5。3例T-LGL病例无CD7表达。3例T-LGL病例进行了TCRα/β检测，全部呈阳性。2例T-PLL小变异型病例也检测到TCRα/β。然而，唯一的PCGDTCL病例也检测到TCRα/β。
成熟的结内T/NK细胞肿瘤较为罕见，表现为白血病的MTNKL更为罕见。不同MTNKL亚型的免疫表型特征存在重叠，评估时需要详细的T/NK细胞检测组合。
