肝脾阿尔法-贝塔 T 细胞淋巴瘤:极具挑战性的诊断实体

IF 1.3 Q4 HEMATOLOGY
A. Gabra, Joanna Polanco, Shrija Thapa, Sumit Sawhney, Alexey Glazyrin
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引用次数: 0

摘要

肝脾T细胞淋巴瘤(HSTCL)是一种罕见的临床侵袭性T细胞淋巴瘤。大多数病例携带γδ T细胞受体(TCR);但在一些更罕见的病例中,肿瘤细胞携带αβ TCR。最近的研究表明,αβ病例可能具有不同的形态学特征,病程也更具侵袭性。在本病例报告中,我们发现αβ型HSTCL与之前的研究结果一致,具有溶血性表现,临床病程非常凶险,且与免疫抑制无关。从形态上看,肿瘤细胞呈弥漫性生长模式,呈泡状形态,在 CD4+ 中小型反应性 T 细胞的背景下呈 CD8+ 阳性。此外,肝脏肿瘤细胞表现为肝门周围定位,而在骨髓中,则发现了emperipolesis的证据。后者可能是所有类型 HSTCL 的全血细胞减少的重要原因。这些不寻常的形态学和临床特征使得诊断这种罕见亚型罕见病非常具有挑战性。需要对更多病例进行分析,以确定αβ/γδ HSTCL在预后或形态上是否是明显不同的实体。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hepatosplenic Alpha-Beta T-Cell Lymphoma: A Challenging Diagnostic Entity
Hepatosplenic T-cell lymphoma (HSTCL) is rare and clinically very aggressive T-cell lymphoma. The majority of cases harbor γδ T-cell receptors (TCRs); however, in some even rarer cases, tumor cells harbor αβ TCR. Recent studies suggest that αβ cases may have distinct morphological characteristics and demonstrate an even more aggressive course. In this case report, we demonstrated that in line with previous findings, αβ case of HSTCL had hemolytic presentation, demonstrated a very aggressive clinical course, and was unrelated to immunosuppression. Morphologically, tumor cells demonstrated diffuse growth pattern, blastoid morphology, and were CD8+ positive on the background of CD4+ small to medium reactive T cells. Additionally, the liver tumor cells demonstrated periportal localization, and in bone marrow, evidence of emperipolesis was noted. The latter finding may significantly contribute to pancytopenia characteristic, all types of HSTCL. Those unusual morphologic and clinical characteristics make diagnosis of this rare subtype of rare disease very challenging. More case analysis is required to establish whether αβ/γδ HSTCL are prognostically or morphologically significantly distinct entities.
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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
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