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首页> 外文期刊>American Journal of Dermatopathology >Epidermotropic B-Cell Lymphoma: A Unique Subset of CXCR3-Positive Marginal Zone Lymphoma
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Epidermotropic B-Cell Lymphoma: A Unique Subset of CXCR3-Positive Marginal Zone Lymphoma

机译：表皮性B细胞淋巴瘤：CXCR3阳性边缘区淋巴瘤的独特子集。

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Background:Epidermotropic B-cell lymphoma is a very rare entity that has primarily been reported in the literature as anecdotal case reports. The majority of the reported cases exhibit a diffuse skin rash affecting middle-aged to older adults with a male predominance. The exact mechanism of marginal zone B-cell localization to the epidermis is unclear.Material and Methods:To describe a very rare subset of cutaneous B-cell lymphoma and explore potential pathogenetic mechanisms for the epidermotropic tendency, the hospital database and literature review was conducted to isolate cases of epidermotropic B-cell lymphoma. Routine hematoxylin and eosin stain followed by selective phenotypic studies.Results:Two of the cases were encountered in the hospital database, whereas 5 cases have been previously reported; material was requested on previously reported cases and was received on 3 of them. In one of the 2 cases encountered in our database, the patient presented with a progressive skin rash over 7 months resembling pityriasis rosea. Subsequent to a diagnosis of B-cell lymphoma, further staging revealed bone marrow involvement. The other patient, an elderly female, presented with isolated nodules. The biopsies of both cases showed areas of superficial band-like lymphocytic infiltration with large monocytoid appearance and an epidermotropic pattern of lymphocyte migration into the epidermis. Neoplastic cells were extensively positive for CD20, CD79a, and BCL-2 and negative for CD10 and BCL-6. Of interest, a similar pityriasis rosea-like presentation was encountered in the cases reported in the literature. All patients were elderly males with established bone marrow, peripheral blood, and spleen involvement several months to years after the initial cutaneous presentation in 3 of them. None of the patients to date have died of lymphoma. CXCR3 epidermotropic B cells were detected in both our cases and in 3 of the 3 previously published cases.Conclusions:Epidermotropic B-cell lymphoma represents a subset of marginal zone lymphoma characterized by a papulosquamous rash most frequently resembling pityriasis rosea, occurring almost exclusively in older males. We speculate that aberrant expression of CXCR3 in marginal zone lymphoma of the skin is associated with migration of lymphoma cells to the epidermis and could lead to an epidermotropic pattern given the known role of CXCR3 expression in neoplastic T cells in the localization of mycosis fungoides to the epidermis. There is a tendency toward bone marrow, spleen, and peripheral blood involvement.
机译：背景：上皮性B细胞淋巴瘤是一种非常罕见的实体，主要在文献中作为传闻病例报道。大多数报道的病例表现出弥漫性皮疹，影响以男性为主的中老年人。材料和方法：为描述皮肤B细胞淋巴瘤的一个非常罕见的子集并探讨表皮趋向性的潜在致病机制，对医院数据库和文献进行了回顾分离出表皮性B细胞淋巴瘤。常规苏木精和曙红染色，然后进行选择性表型研究。结果：医院数据库中遇到了2例病例，而先前已经报道了5例病例。要求对以前报告的案件提供材料，其中3个收到。在我们数据库中遇到的2例病例之一中，患者在7个月内出现了类似玫瑰糠疹的进展性皮疹。诊断为B细胞淋巴瘤后，进一步分期显示骨髓受累。另一名患者，一名老年女性，出现孤立的结节。两种病例的活检均显示表面带状淋巴细胞浸润区域，具有大的单核细胞样外观以及淋巴细胞向表皮迁移的表皮模式。肿瘤细胞对CD20，CD79a和BCL-2广泛呈阳性，而对CD10和BCL-6呈阴性。有趣的是，文献报道的病例中也遇到了类似的糠疹性玫瑰花样症状。所有患者均为年龄较大的男性，其中3例在首次皮肤表现后数月至数年已确诊骨髓，外周血和脾脏受累。迄今为止，没有患者死于淋巴瘤。结论：上皮性B细胞淋巴瘤是边缘区淋巴瘤的一部分，其特征是丘疹性皮疹，最常见于玫瑰糠疹，几乎只发生在老年患者中。男性。我们推测，在皮肤边缘区淋巴瘤中CXCR3的异常表达与淋巴瘤细胞向表皮的迁移有关，并且鉴于已知的肿瘤性T细胞中CXCR3表达在真菌病真菌定位中的作用，可能导致表皮型。表皮。有骨髓，脾脏和外周血受累的趋势。

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