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骨髓中胸腺星状 B 细胞微环境相关的 T 细胞淋巴母细胞白血病/淋巴瘤。

T cell lymphoblastic leukaemia/lymphoma associated with a microenvironment of thymic asteroid B cells in the bone marrow.

机构信息

Departments of Histopathology and Haematology, Imperial College Healthcare NHS Trust, London, UK.

出版信息

Histopathology. 2010 Oct;57(4):549-54. doi: 10.1111/j.1365-2559.2010.03663.x. Epub 2010 Sep 28.

Abstract

AIMS

Asteroid B cells are a component of normal thymus. It is currently unclear whether these cells are identifiable in T cell lymphoblastic leukaemia/lymphoma (T-ALL/LBL) of the thymus. The aim of this study was to identify asteroid B cells both in thymic and extrathymic tissue involved by T-ALL/LBL.

METHODS AND RESULTS

Thymic, lymph node (LN) and bone marrow trephine biopsy (BMTB) samples from eight patients with T-ALL/LBL were reviewed. All had been investigated by immunohistochemistry and one by fluorescent in situ hybridization (FISH). The BMTB samples of two of eight T-ALL/LBLs and LN sample in one of them showed the presence of asteroid-shaped B cells with dendritic cytoplasmic processes. These B cells also expressed CD23 and the features were akin to the unique thymic asteroid B cells. Both patients had aggressive/resistant disease. Cytogenetic analysis in one showed a complex translocation involving the T cell receptor beta (TCRB) gene at 7q35 and a distal region of 9q known to harbour the NOTCH1 gene.

CONCLUSION

This is the first report of T-ALL/LBL documenting the presence of an asteroid B cell-rich microenvironment at bone marrow and LN sites. In this small subset, T-ALL/LBL cells are possibly dependent upon asteroid B cells, and whether targeting of asteroid B cells with anti-CD20 monoclonal antibody in such cases will result in clinical benefit remains to be determined.

摘要

目的

星型 B 细胞是正常胸腺的组成部分。目前尚不清楚这些细胞是否能在胸腺 T 细胞淋巴母细胞白血病/淋巴瘤(T-ALL/LBL)中识别。本研究旨在鉴定 T-ALL/LBL 累及的胸腺和胸腺外组织中的星型 B 细胞。

方法和结果

对 8 例 T-ALL/LBL 患者的胸腺、淋巴结(LN)和骨髓活检(BMTB)样本进行了回顾性分析。所有患者均进行了免疫组织化学检查,其中 1 例进行了荧光原位杂交(FISH)检查。8 例 T-ALL/LBL 中有 2 例 BMTB 样本和其中 1 例的 LN 样本中存在具有树突状细胞质突起的星型 B 细胞。这些 B 细胞还表达 CD23,其特征与独特的胸腺星型 B 细胞相似。这两个患者均患有侵袭性/耐药性疾病。其中一个患者的细胞遗传学分析显示涉及 7q35 处 T 细胞受体β(TCRB)基因和已知含有 NOTCH1 基因的 9q 远端区域的复杂易位。

结论

这是首例报道 T-ALL/LBL 在骨髓和 LN 部位存在富含星型 B 细胞的微环境。在这一小部分患者中,T-ALL/LBL 细胞可能依赖于星型 B 细胞,而在这种情况下,用抗 CD20 单克隆抗体靶向星型 B 细胞是否会带来临床获益仍有待确定。

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