Li Meng-Fang, Hsiao Cheng-Hsiang, Chen Yi-Lin, Huang Wen-Ya, Lee Yi-Hsuan, Huang Hsien-Neng, Lien Huang-Chun
Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan.
J Cutan Pathol. 2012 Feb;39(2):274-8. doi: 10.1111/j.1600-0560.2011.01814.x. Epub 2011 Dec 27.
Primary effusion lymphoma, a human herpesvirus 8 (HHV8)-associated lymphoma, is uncommon, and it is usually seen in human immunodeficiency virus (HIV)-infected patients. It presents as a body cavity-based lymphomatous effusion, but several cases of the so-called solid primary effusion lymphoma presenting as solid tumors without associated lymphomatous effusion have been reported. They have similar clinical, histopathological and immunophenotypical features. Most of them have a B-cell genotype. This suggests the solid variant may represent a clinicopathological spectrum of primary effusion lymphoma. We report a case of HHV8-associated lymphoma histopathologically and immunophenotypically mimicking cutaneous anaplastic large cell lymphoma. The patient was a 31-year-old HIV-seropositive man presenting with skin nodules over his right thigh. Biopsy of the nodules showed anaplastic large cells infiltrating the dermis. These malignant cells strongly expressed CD3, CD30 and CD43. Cutaneous anaplastic large T-cell lymphoma was initially diagnosed, but further tests, including immunoreactivity for HHV8 protein and clonal rearrangements of immunoglobulin genes, confirmed the diagnosis of HHV8-associated B-cell lymphoma with aberrant T-cell marker expression. This case provides an example of solid primary effusion lymphoma mimicking cutaneous anaplastic large T-cell lymphoma and highlights the importance of HHV8 immunohistochemistry and molecular tests in the diagnosis of HHV8-associated lymphoma with a cutaneous presentation.
原发性渗出性淋巴瘤是一种与人类疱疹病毒8型(HHV8)相关的淋巴瘤,较为罕见,通常见于人类免疫缺陷病毒(HIV)感染患者。它表现为以体腔为基础的淋巴瘤性渗出液,但也有几例所谓的实体性原发性渗出性淋巴瘤的报道,这些病例表现为实体瘤,无相关的淋巴瘤性渗出液。它们具有相似的临床、组织病理学和免疫表型特征。其中大多数具有B细胞基因型。这表明实体变体可能代表原发性渗出性淋巴瘤的临床病理谱。我们报告一例HHV8相关淋巴瘤,其组织病理学和免疫表型类似于皮肤间变性大细胞淋巴瘤。患者为一名31岁的HIV血清阳性男性,右大腿出现皮肤结节。结节活检显示间变性大细胞浸润真皮。这些恶性细胞强烈表达CD3、CD30和CD43。最初诊断为皮肤间变性大T细胞淋巴瘤,但进一步检查,包括HHV8蛋白免疫反应性和免疫球蛋白基因的克隆重排,证实诊断为伴有异常T细胞标志物表达的HHV8相关B细胞淋巴瘤。该病例提供了一个实体性原发性渗出性淋巴瘤模仿皮肤间变性大T细胞淋巴瘤的例子,并强调了HHV8免疫组织化学和分子检测在诊断具有皮肤表现的HHV8相关淋巴瘤中的重要性。