Awaya Norihiro, Mori Shigehisa, Takeuchi Hitoshi, Mori Shigeo, Sugano Yasuo, Kamata Tamihiro, Takeuchi Tsutomu, Abe Toru
The Second Department of Internal Medicine, Saitama Medical Center, Saitama Medical School, Saitama, Japan.
Am J Hematol. 2002 Mar;69(3):200-4. doi: 10.1002/ajh.10059.
The t(2;5)(p23;q35) translocation results in the formation of a unique chimeric NPM-ALK protein (p80). Expression of this protein is considered to be one of the clinical features of anaplastic large cell lymphoma (ALCL). Recently recognized as one clinical subtype of ALCL, the small cell variant is prone to have a leukemic presentation. Although the small cell variant has been recognized as a subtype of ALCL, the clinical properties of this subtype, especially the immunophenotype of lymphoma cells in peripheral blood, have not yet been fully described. This report shows that neither CD30 nor p80 is detected by immunostaining in the predominant small cell malignant clone and also in large lymphoma cells in peripheral blood, while large cells and occasionally observed small cells in bone marrow were found to be positive for CD30 and p80. Our findings suggest that differential expression of CD30 and p80 between peripheral blood and bone marrow lymphoma cells is a property of the small cell variant of ALCL.
t(2;5)(p23;q35)易位导致形成一种独特的嵌合NPM-ALK蛋白（p80）。该蛋白的表达被认为是间变性大细胞淋巴瘤（ALCL）的临床特征之一。小细胞变异型最近被确认为ALCL的一种临床亚型，易于出现白血病表现。尽管小细胞变异型已被确认为ALCL的一种亚型，但该亚型的临床特性，尤其是外周血中淋巴瘤细胞的免疫表型，尚未得到充分描述。本报告显示，在主要的小细胞恶性克隆以及外周血中的大淋巴瘤细胞中，免疫染色均未检测到CD30和p80，而骨髓中的大细胞以及偶尔观察到的小细胞被发现CD30和p80呈阳性。我们的研究结果表明，外周血和骨髓淋巴瘤细胞之间CD30和p80的差异表达是ALCL小细胞变异型的一个特性。
