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ALK 阳性髓外浆细胞瘤,表达 CLTC-ALK 融合转录本。

ALK-positive extramedullary plasmacytoma with expression of the CLTC-ALK fusion transcript.

机构信息

Department of Pathology, West China Hospital of Sichuan University, Chengdu, China.

出版信息

Pathol Res Pract. 2011 Sep 15;207(9):587-91. doi: 10.1016/j.prp.2011.07.001. Epub 2011 Aug 19.

Abstract

Anaplastic lymphoma kinase (ALK)-positive non-Hodgkin lymphoma (NHL) was long thought to be a disease occurring uniquely in T or null-cell lymphomas. More recently, however, a small number of B-lineage lymphomas have been reported to express ALK fusion genes. These tumors often exhibit a plasmablastic morphology, a finding which prompted our interest in looking for ALK fusions in plasma cell neoplasms. We studied 46 cases of extramedullary plasmacytoma by immunostaining with anti-ALK antibody and fluorescence in situ hybridization (FISH) analysis using an ALK break-apart probe and found one case to be ALK protein-positive and demonstrated the disruption of the ALK gene in this case. Immunohistochemistry showed that the tumor cells were strongly positive for CD138, VS38c, and epithelial membrane antigen, but lacked expression of CD20, CD79a, CD45, and CD30. Both RT-PCR and genomic DNA-PCR confirmed the CLTC-ALK fusion. This finding expands the lists of the ALK-positive tumors, and ALK-positive extramedullary plasmacytoma may benefit from the treatment of ALK inhibitor in the future.

摘要

间变性淋巴瘤激酶(ALK)阳性非霍奇金淋巴瘤(NHL)曾被认为是一种仅发生于 T 细胞或无细胞淋巴瘤的疾病。然而,最近有少数 B 细胞淋巴瘤被报道表达 ALK 融合基因。这些肿瘤通常表现为浆母细胞形态,这一发现促使我们对浆细胞肿瘤中 ALK 融合进行研究。我们通过免疫组织化学染色用抗 ALK 抗体和荧光原位杂交(FISH)分析研究了 46 例髓外浆细胞瘤,使用 ALK 断裂探针,发现 1 例为 ALK 蛋白阳性,并在该病例中显示 ALK 基因的断裂。免疫组化显示肿瘤细胞强烈表达 CD138、VS38c 和上皮膜抗原,但缺乏 CD20、CD79a、CD45 和 CD30 的表达。RT-PCR 和基因组 DNA-PCR 均证实存在 CLTC-ALK 融合。这一发现扩展了 ALK 阳性肿瘤的列表,ALK 阳性髓外浆细胞瘤将来可能受益于 ALK 抑制剂的治疗。

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