Division of Haematology, McGill University Health Centre, Montreal, Quebec, Canada.
Infectious Disease and Immunity in Global Health Program, Research Institute of the McGill University Health Centre, Montréal, Quebec, Canada.
BMJ Case Rep. 2021 Jan 28;14(1):e238644. doi: 10.1136/bcr-2020-238644.
An 88-year-old Inuit man from Northern Canada presented with an extensive skin rash associated with numerous violaceous skin nodules on his palms and lower extremities. Biopsy of a skin nodule revealed Kaposi's sarcoma (KS), a human herpesvirus 8 (HHV8)-associated malignancy, whereas biopsy of the erythematous skin showed an atypical infiltrate of CD4-positive T-cells that, together with TCR gene rearrangement and presence of clonal T-cells in peripheral blood by flow cytometry, was consistent with a T-cell lymphoma, mycosis fungoides (MF) subtype. Serology was negative for HIV and HTLV-I/II and no immunodeficiency syndrome was identified. The patient was successfully treated with an oral retinoid for KS, and with topical hydrocortisone and ultraviolet B (UVB) phototherapy for MF. This case highlights the existence of HHV8-related lesions in native persons of Northern Canada, and also that MF-induced immunosuppression combined with immunosenescence may play a role in the development of non-HIV-related KS.
一位来自加拿大北部的 88 岁因纽特男子出现广泛的皮疹，并伴有手掌和下肢多处紫红色皮肤结节。皮肤结节活检显示卡波西肉瘤（KS），一种人类疱疹病毒 8（HHV8）相关的恶性肿瘤，而红斑皮肤活检显示 CD4 阳性 T 细胞的非典型浸润，与 TCR 基因重排以及流式细胞术检测到外周血中的克隆 T 细胞一致，符合 T 细胞淋巴瘤，蕈样肉芽肿（MF）亚型。血清学检查 HIV 和 HTLV-I/II 均为阴性，未发现免疫缺陷综合征。该患者成功地接受了口服维甲酸治疗 KS，以及局部氢化可的松和紫外线 B（UVB）光疗治疗 MF。本病例强调了加拿大北部原住民中存在 HHV8 相关病变，并且 MF 诱导的免疫抑制与免疫衰老可能在非 HIV 相关 KS 的发展中起作用。
