皮肤CD8^+嗜表皮性细胞毒性T细胞淋巴瘤伴侵袭性病程(法语)[法语论文]

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摘  要:背景:皮肤CD8^+嗜表皮性细胞毒性T细胞淋巴瘤是一种近来报道的罕见的原发性皮肤淋巴瘤,法语论文范文,临床表现具有侵袭性。文献中仅有约20例报道。作者在此报道了1例罕见的伴发皮肤血管炎和淋巴组织增生的患者。病例报道:1例42岁的塞内加尔男性患者因其皮损表现为结节且迅速蔓延并发生坏死和溃疡而入院,患者近期还发生体重减轻、发热以及多发性淋巴结增大。皮肤组织学略论显示少见的血管中心性间质和大CD8^+细胞毒性T细胞嗜表皮性真皮浸润,伴有皮肤血管炎和纤维素样坏死,联合化疗4个月后患者死亡。讨论:此患者的特点符合Berti所描述的原发性皮肤CD8^+嗜表皮性细胞毒性T细胞淋巴瘤。多数本病患者的临床表现为结节和溃疡,组织学多表现为皮肤组织的多形性淋巴细胞浸润伴明显而持久的嗜表皮性。免疫组化检查显示淋巴细胞表型为CD8^+并且表达细胞毒素蛋白质,这可能解释了本病的局部和系统的侵袭性病变及浸润和坏死皮损血管损坏的实质。Background. Cutaneous CD8 +epidermotropic cytotoxic T-cell lymphoma is a recently described rare primary cutaneous lymphoma exhibiting aggressive clinical behavior. Only about twenty cases have been described in the literature. Below we report a case involving unusual association of cutaneous vasculitis and lymphoproliferation. Case report. A 42-year-old senegalese man was hospitalized for cutaneous nodular lesions, which rapidly spread and became necrotic and ulcerated, he had recent weight loss with fever and multiple enlarged lymph nodes. Cutaneous histological analysis showed epidermotropic dermal infiltrate comprising medium and large cd8 + cytotoxic t-cells of unusual angiocentricity with cutaneous vasculitis and fibrinoid necrosis, the patient died 4 months after initiation of treatment with multi-agent chemotherapy. Discussion. This patient presented the characteristics of primary Cutaneous CD8 + epidermotropic cytotoxic T-ceU lymphoma described by Berti. The clinical findings in most cases consist of nodular and ulcerative cutaneous lesions. Histologically, the cutaneous infiltrate is composed of pleomorphic lymphocytes with marked and constant epidermotropism. Immunohistochemistry shows lymphocytes expressing a CD8 + phenotype and cytotoxic proteins, which probably accounts for the local and systemic aggressiveness of the disease, as well as the angio destructive nature of the infiltrate and the necrotic lesions.

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