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惰性 T 细胞大颗粒淋巴细胞白血病造血干细胞移植后:临床病理和分子分析。

Indolent T-cell large granular lymphocyte leukaemia after haematopoietic SCT: a clinicopathologic and molecular analysis.

机构信息

Department of Medicine, Queen Mary Hospital, Hong Kong, China.

出版信息

Bone Marrow Transplant. 2012 Jul;47(7):952-6. doi: 10.1038/bmt.2011.212. Epub 2011 Oct 31.

DOI:10.1038/bmt.2011.212
PMID:22041849
Abstract

Four women and three men after allogeneic (n=4) and autologous (n=3) haematopoietic SCT (HSCT) were observed to have an increase in T-cell large granular lymphocytes (T-LGLs) of CD3+CD8+ phenotype for a median of 41 (15-118) months. Clonal rearrangement of the T-cell receptor gene was verified by two PCR techniques and direct DNA sequencing, confirming that the cases were neoplastic and therefore classifiable as T-LGL leukaemia. In the allogeneic HSCT cases, T-LGL leukaemia was derived from donor T cells in three patients, as shown by DNA chimerism analysis, and recipient T cells in one patient who had graft failure previously. None of the patients showed cytopenia, autoimmune phenomenon or organ infiltration, which were features typical of de novo T-LGL leukaemia. Six patients had remained asymptomatic with stable large granular lymphocyte counts. One patient died from cerebral relapse of the original lymphoma. T-LGL leukaemias occurring post-HSCT are distinct from de novo T-LGL leukaemia and may have a different pathogenesis and clinical course. Patients did not require specific treatment, and the disease remained stable for long periods.

摘要

4 名接受异基因(n=4)和自体(n=3)造血干细胞移植(HSCT)的女性和 3 名男性患者观察到 CD3+CD8+表型的 T 细胞大颗粒淋巴细胞(T-LGL)数量增加,中位数为 41(15-118)个月。通过两种 PCR 技术和直接 DNA 测序证实 T 细胞受体基因的克隆重排,证实这些病例是肿瘤性的,因此可归类为 T-LGL 白血病。在异基因 HSCT 病例中,T-LGL 白血病来源于 3 名患者的供体 T 细胞,如 DNA 嵌合体分析所示,1 名患者先前发生移植物衰竭,来源于受体 T 细胞。所有患者均无血细胞减少症、自身免疫现象或器官浸润,这些都是新发 T-LGL 白血病的典型特征。6 名患者无症状,大颗粒淋巴细胞计数稳定。1 名患者死于原发性淋巴瘤的脑复发。HSCT 后发生的 T-LGL 白血病与新发 T-LGL 白血病不同,可能具有不同的发病机制和临床过程。患者不需要特定的治疗,疾病长期稳定。

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