Primary Cutaneous T-Cell Lymphoma with CD8 Immunophenotype and Angiocentric and Angiodestructive Features -A Case Report-

原發性皮膚T細胞淋巴癌合併CD8免疫表型及血管中心性和血管破壞性之病理變化 –病例報告–

郭宏文(Hung-Wen Kuo) ； 邱耀堃(Yao-Kun Chiu) ； 何宜承(Ji-chen Ho) ； 吳鴻誠(Hong-cheng Wu)

《中華皮膚科醫學雜誌》 18卷2期 (2000/06) Pp. 105-113

摘要

吾人在此報告一例原發性皮膚T細胞淋巴癌合併CD8免疫表型及血管中心性和血管破壞性病理變化之病例。這是一位49歲台灣籍男子，在入院前四個月，身上開始出現廣泛分佈的暗紅色到紫色的丘疹、斑塊，以及潰瘍或結痂的腫塊，並有少許水泡病灶。住院時，從水泡病灶所作的切片檢查，初步診斷為非典型多形性紅斑；但是在靜脈給予類固醇後，皮膚病灶仍持續擴展。住院後一個月，吾人從新生的丘疹及原潰瘍斑塊處再作切片檢查，二處切片病理均顯示異常淋巴細胞親皮浸潤及Pautrier微膿瘍形成；在上層至中層真皮，則有血管周圍及血管內的異常淋巴細胞浸潤。免疫表型檢查發現這些細胞為CD3陽性、CD4陰性、CD8陽性、CD30陰性、CD56陰性。EBER1原位雜合檢查為陰性反應。詳細檢查並無發現皮膚以外病灶。於是我們診斷此為一發展迅速型的原發性CD8陽性皮膚T細胞淋巴癌，並給予化學治療及干擾素治療。據吾人所知，在文獻上這是第二例原發性CD8陽性皮膚T細胞淋巴癌，同時合併親皮浸潤、Pautrier微膿瘍及血管中心性和血管性之病理變化。

並列摘要

We herein report a case of primary cutaneous T-cell lymphoma (CTCL) with CD8 immunophenotype and angiocentric and angiodestructive features. This patient was a 49-year-old Taiwanese man, who had gradually developed widespread dark red to purpuric papules and plaques, ulcerated or crusted noduloplaques and a few blisters 4 months prior to admission. Skin biopsy from one blister on admission was initially diagnosed as atypical erythema multiforme; however, the skin lesions progressed despite intravenous hydrocortisone therapy. Further skin biopsies taken from one newly developed papule and one old ulcerated plaque showed atypical lymphoid cell epidermotropism with formation of Pautrier’s microabscesses and perivascular and intravascular infiltration of atypical lymphocytes in the upper to middle dermis. Immunophenotypically, these atypical lymphocytes were CD3+, CD4-, CD8+, CD30- and CD56-. In situ hybridization study with EBER1 probe was negative. There was no evidence of extracutaneous involvement. The diagnosis of rapidly progressive primary CD8-positive CTCL was made and combination chemotherapy with interferon- α was given. To our knowledge, this case is the second one in the literature in which CD8-positive CTCL showed epidermotropism, Pautrier’s microabscesses and angiocentric and angiodestructive infiltration.

並列關鍵字

cutaneous T-cell lymphoma ； CD8 phenotype ； Angiocentric and angiodestructive ； Interferon-α ； Anti-intercellular substance antibody