Wang Lei, Li Chengxin, Gao Tianwen
Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xian, China.
J Cutan Pathol. 2011 Feb;38(2):221-6. doi: 10.1111/j.1600-0560.2010.01538.x.
Intravascular lymphoma (IL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47-year-old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype.
血管内淋巴瘤（IL）是一种罕见的非霍奇金淋巴瘤变体，好发于皮肤。大多数报道的病例为大B细胞淋巴瘤。血管内间变性大细胞淋巴瘤（IALCL）极为罕见。对1例皮肤IALCL病例进行了回顾性分析。分析了苏木精-伊红染色切片和免疫组化染色结果。患者为一名47岁女性，其背部出现多个红斑和斑块。病变对CHOP（环磷酰胺、羟基柔红霉素、长春新碱、泼尼松）化疗反应良好，但治疗后不久复发。该患者带瘤生存8年，但最终失访。组织病理学上，肿瘤从IL演变为血管外淋巴瘤。这在疾病不同阶段获取的活检中得到了证实。淋巴瘤细胞CD30、CD45、CD3、CD4、CD5和Ki67染色呈阳性，且缺乏间变性淋巴瘤激酶（ALK）、CD8、CD45RA、CD45RO、CD20、CD79、CD56、穿孔素和颗粒酶B的表达。我们的结果表明，IALCL代表IL的一种独特亚型，在组织病理学和生物学上与具有B、NK或T细胞表型的IL不同。
