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原发性皮肤免疫细胞瘤。一种容易被误诊为皮肤淋巴细胞增生症的B细胞淋巴瘤。

Primary cutaneous immunocytoma. A B-cell lymphoma that can easily be mistaken for cutaneous lymphoid hyperplasia.

作者信息

LeBoit P E, McNutt N S, Reed J A, Jacobson M, Weiss L M

机构信息

Department of Pathology, University of California, San Francisco 94143-0506.

出版信息

Am J Surg Pathol. 1994 Oct;18(10):969-78. doi: 10.1097/00000478-199410000-00001.

Abstract

It has been estimated that immunocytomas comprise roughly 2% of all cutaneous lymphomas. We studied five patients with primary cutaneous immunocytomas who presented with cutaneous nodules or plaques. Many of the infiltrates were "top-heavy" and polymorphous with admixed eosinophils, macrophages, lymphoid follicles, and non-neoplastic lymphocytes. Other potentially confusing findings were one case each of spongiotic dermatitis and leukocytoclastic vasculitis. The neoplastic cells were often situated at the peripheries of nodules and ranged from those with nuclei that resembled small lymphocytes to others that resembled immunoblasts. Most had eccentrically placed nuclei and fan-shaped cytoplasm. Monotypic kappa-light chain was found in all five cases, accompanied by gamma-heavy chain in two cases, and mu-heavy chain in one. In situ hybridization detected only kappa-mRNA in the four cases that yielded technically satisfactory results. The neoplastic cells did not express the B-cell antigen CD20; T-cells formed the centers of many nodules. Inappropriate staining for CD43 was evident in the neoplastic cells of one case. Because of reports of immunocytomas complicating acrodermatitis chronica atrophicans, we stained sections with an antiserum to Borrelia burgdorferi, which did not detect that organism. In situ hybridization did not detect EBER-1 RNA of the Epstein-Barr virus, which can be present in immunocytomas in immunocompromised patients. One patient died of disease after failing chemotherapy; another is alive with disseminated disease, and three are in remission following excision of lesions alone in two patients and chemotherapy in one patient who had relapsed following both excision and radiation therapy.

摘要

据估计,免疫细胞瘤约占所有皮肤淋巴瘤的2%。我们研究了5例原发性皮肤免疫细胞瘤患者,这些患者表现为皮肤结节或斑块。许多浸润灶呈“顶部较重”且多形性,伴有嗜酸性粒细胞、巨噬细胞、淋巴滤泡和非肿瘤性淋巴细胞混合。其他可能造成混淆的表现分别为1例海绵状皮炎和1例白细胞破碎性血管炎。肿瘤细胞常位于结节周边,细胞核形态多样,从小淋巴细胞样到免疫母细胞样。大多数细胞核偏位,细胞质呈扇形。所有5例均发现单克隆kappa轻链,2例伴有gamma重链,1例伴有mu重链。在4例技术结果满意的病例中,原位杂交仅检测到kappa - mRNA。肿瘤细胞不表达B细胞抗原CD20;T细胞构成许多结节的中心。1例患者的肿瘤细胞中CD43染色异常。由于有免疫细胞瘤并发慢性萎缩性肢端皮炎的报道,我们用抗伯氏疏螺旋体抗血清对切片进行染色,但未检测到该病原体。原位杂交未检测到爱泼斯坦 - 巴尔病毒的EBER - 1 RNA,该病毒可存在于免疫功能低下患者的免疫细胞瘤中。1例患者化疗失败后死于疾病;另1例患者患有播散性疾病存活,2例患者仅通过切除病变缓解,1例患者在切除和放疗后复发,经化疗后缓解。

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