Intriago B Marvila, Peña O Camila, Gray H Ana María, Cabrera C María Elena, Martínez C Virginia
Sección Hematología, Hospital del Salvador, Santiago, Chile.
Rev Med Chil. 2012 Mar;140(3):368-72. doi: 10.4067/S0034-98872012000300014.
Primary cutaneous aggressive epidermotropic cytotoxic CD8 positive T cell lymphoma, is an uncommon disease, with an aggressive clinical behavior. Differentiation with other types of cutaneous T-cell lymphoma (CTCL) that express a CD8+ cells, is based only on clinical grounds and in certain morphological characteristics, such as a marked epidermotropism with squamous cell necrosis. We report a 50-year-old male presenting with painless cutaneous lesions appearing in trunk, limbs, scalp and face, suggestive of cutaneous lymphoma. He was admitted to the hospital in bad conditions, with confluent papules and tumors, some of them ulcerated and with foul smelling honey-colored crusts, involving the complete body surface. Cutaneous biopsy demonstrated a CD8 positive epidermotropic cytotoxic T cell lymphoma. He was treated with chemotherapy with an excellent initial response, but cutaneous lesions reappeared after four cycles. He did not respond to rescue chemotherapy and died seven months after diagnosis.
原发性皮肤侵袭性亲表皮细胞毒性CD8阳性T细胞淋巴瘤是一种罕见疾病,具有侵袭性临床行为。与其他表达CD8 +细胞的皮肤T细胞淋巴瘤(CTCL)的鉴别仅基于临床依据和某些形态学特征,例如伴有鳞状细胞坏死的显著亲表皮性。我们报告了一名50岁男性,其躯干、四肢、头皮和面部出现无痛性皮肤病变,提示皮肤淋巴瘤。他入院时病情严重,有融合性丘疹和肿瘤,其中一些发生溃疡并有恶臭的蜜色痂皮,累及全身体表。皮肤活检显示为CD8阳性亲表皮细胞毒性T细胞淋巴瘤。他接受了化疗,初始反应良好,但四个周期后皮肤病变复发。他对挽救性化疗无反应,诊断后七个月死亡。