Generalized Edema and Telangiectasia: Unusual Presentation of Intravascular Large B-cell Lymphoma

以全身水腫及血管擴張為皮膚表現的罕見血管內大型B細胞淋巴瘤

高嘉懋(Chia-Mao Kao) ； 張英睿(Ying-Jui Chang) ； 蕭正祥(Cheng-Hsiang Hsiao) ； 徐毓聆(Yu-Ling Hsu) ； 王秉槐(Ping-Huai Wang) ； 蔡宗樺(Tsung-Hua Tsai) ； 蘇琳惠(Lim-Hui Su)

《中華皮膚科醫學雜誌》 27卷4期 (2009/12) Pp. 248-253

https://doi.org/10.29784/DS.200912.0007

摘要

血管內B型大淋巴球淋巴瘤是一種在小血管內充滿大型腫瘤性B細胞的淋巴瘤，最常表現在真皮層及皮下組織的小血管。除非進展到疾病後期，否則一般不會有淋巴結或其他器官實質的侵犯。臨床表現非常多樣化，是由於腫瘤細胞容易侵犯各器官內小血管的內皮細胞所致。我們報告一個五十一歲的男性，其臨床表現為發燒、意識改變、全身性水腫、以及顯著的微血管擴張。我們在其右側大腿的病灶施行皮膚切片。病理下可見表淺真皮層有擴張的血管以及深部真皮層的小血管內充滿大型的腫瘤性淋巴球。免疫化學染色呈現CD45及CD79a陽性反應，進一步證實其為B淋巴球。這是血管內B型大淋巴球淋巴瘤一個罕見的病例，在皮膚上的表現只有全身性水腫及血管擴張。

並列摘要

Intravascular large B-cell lymphoma is characterized by clusters of large neoplastic B cells within small blood vessels, most commonly in the dermis and subcutis. There is no nodal or parenchymal involvement until late in the disease, and its endothelial tropism is responsible for the variable clinical features. We report the case of a 51 year-old man who presented with fever, altered mental status, generalized edema, and profound telangiectasia. The diagnosis was made by a skin biopsy from the right thigh. Histology revealed ectatic vessels in the superficial dermis and large atypical lymphoid cells filling the small blood vessels in the deep dermis. Immunohistochemical staining showed strongly positive results for CD45 and CD79a. This was a rare case of intravascular large B-cell lymphoma with generalized edema and telangiectasia as the only cutaneous manifestation.

並列關鍵字

Intravascular large B-cell lymphoma ； Angiotropic lymphoma ； Telangiectasia