原发性皮肤边缘区B细胞淋巴瘤

Primary cutaneous marginal zone B-cell lymphoma: a case report

报告1例原发性皮肤边缘区B细胞淋巴瘤(PCMZL)。患者男,26岁。右大腿伸侧、左肩部及左前臂各一红色或紫红色浸润性斑块,大小不等,界限清楚,表面可见米粒大丘疹,无鳞屑、破溃及压痛。皮损组织病理检查:真皮层可见淋巴样细胞弥漫或结节状浸润,少量累及表皮,浸润细胞中大量小至中等大小的中心细胞样淋巴细胞,胞质透亮,可见轻度核异形;表皮下、浸润细胞周围偶见浆细胞。免疫组化:可见大量反应性的CD3+T细胞,其周围环绕的小淋巴细胞CD20、CD79a及B细胞淋巴瘤/白血病因子(Bcl)-2均(+);肿瘤细胞增殖核抗原(Ki-67)(>40%+);浆细胞限制性表达轻链-λ,不表达轻链-κ;散在肿瘤细胞CD30、CD21、CD10、Bcl-6及D型细胞周期蛋白(cyclin D)1均(+)。诊断:PCMZL。治疗:予手术切除右大腿伸侧及左肩部皮损,术后随访至今,皮损未复发及未见新发皮损。

A case of primary cutaneous marginal zone B-cell lymphoma（PCMZL） is reported. A 26-year-old man presented with three demarcated erythematous infiltrated plaques on the right thigh, left shoulder and forearm in various sizes, with grain-size papules on the surface, but no scales, ulceration, or tenderness. Histopathological examination showed diffuse or nodular infiltration of lymphoid cells in the dermis and part of the epidermis. The infiltrated cells included small to medium sized centrocyte-like lymphocytes with slightly irregular nuclei and clear cytoplasm. Plasma cells presented beneath the epidermis or around the infiltrated cells. Immunoehemical staining revealed CD3-positive T cells surrounded by CD20, CD79a and Bcl-2-positive B cells. Ki-67 was positive in approximately 40% of the tumor cells. Tumor cells only expressed immunoglobulin lambda light chain, but not Kappa light chain. Only sparse cells were positive for CD30, CD10, CD21, Bcl-6 and cyclinD1. The diagnosis of PCMZL was made. The patient was treated with surgical excisions of lesions on the right thigh and left shoulder. There was no recurrence or evidence of new lesion during the follow-up period.