Rieger K E, Polidore T, Warnke R, Kim J
Department of Dermatology, Stanford University, Stanford, CA 94305, USA.
J Cutan Pathol. 2011 Feb;38(2):216-20. doi: 10.1111/j.1600-0560.2010.01528.x.
Systemic cases of the CD30-positive T-cell neoplasm, anaplastic large cell lymphoma (ALCL), are typically anaplastic lymphoma kinase (ALK)-positive. The failure to express ALK protein has been shown to portend a worse prognosis. We describe a case of ALK-negative systemic ALCL that presented as a violaceous plaque on the scalp of a 79-year-old man. Interestingly, the neoplastic cells were confined largely within vascular spaces, a configuration that is exceedingly rare in the skin and is more typically seen with intravascular large B-cell lymphoma. In addition, bcl-2 immunohistochemical staining was strongly positive in this case, which may portend a more aggressive clinical course. To our knowledge, this report represents the first case of an ALK-negative ALCL to present intravascularly in the skin. Therefore, the recognition of systemic anaplastic T-cell lymphoma present within the intravascular spaces is important to avoid misdiagnosis.
CD30阳性T细胞肿瘤——间变性大细胞淋巴瘤（ALCL）的系统性病例通常是间变性淋巴瘤激酶（ALK）阳性。已证明ALK蛋白表达缺失预示着更差的预后。我们描述了一例ALK阴性的系统性ALCL病例，该病例表现为一名79岁男性头皮上的紫罗兰色斑块。有趣的是，肿瘤细胞主要局限于血管腔内，这种形态在皮肤中极为罕见，更常见于血管内大B细胞淋巴瘤。此外，该病例中bcl-2免疫组化染色呈强阳性，这可能预示着更具侵袭性的临床病程。据我们所知，本报告代表了首例血管内呈现的ALK阴性ALCL病例。因此，识别血管腔内存在的系统性间变性T细胞淋巴瘤对于避免误诊很重要。
