胚母自然殺手細胞淋巴癌是一種臨床上進展迅速的血液腫瘤同時有很高的比率會有皮膚侵犯。本文報告一例19歲男性病人在臉上、軀幹、及四肢出現無症狀紅色浸潤性大小不一的板塊及結節。皮膚切片檢體顯示有中等大小多型性具有胚母細胞型態的細胞在真皮層浸潤。免疫組織染色顯示腫瘤細胞對於CD56、CD43、CD123、及末端去氧核酸轉移脢的染色呈陽性反應。腫瘤細胞對於B細胞、T細胞、及骨髓細胞抗原標記均為陰性。原位雜交方法無法偵測到EB病毒的RNA。抽出骨髓用流式細胞儀分析,其中的胚母細胞多為CD56陽性的。病人被診斷為胚母自然殺手細胞淋巴癌,並接受L-asparaginase為基礎的化學治療(L-asparaginase、cyclophamide、etoposide)病人在接受兩次的化學治療之後皮膚病灶已經完全消失。
Blastic natural killer (NK)-cell lymphoma is a clinically aggressive hematologic neoplasm with a high incidence of cutaneous involvement. We report a 19-year-old man who presented with asymptomatic, erythematous, infiltrated bean plaques and nodules of varioussizes, scattered over the face, trunk and extremities. A skin biopsy specimen revealed diffuse dermal infiltration of medium-sized pleomorphic cells with a blastic appearance. Immunohistochemical studies showed that the tumor cells were positive for CD56, CD43, CD123, and terminal deoxynucleotidyl transferase (TdT). The neoplastic cells were negative for B-cell, T-cell, and myeloid cell markers. In situ hybridization for Epstein-Barr virus encoded RNA(EBER) gave a negative result. Flow cytometric analysis of bone marrow aspirate demonstrated a CD56 positive population of blastic cells. A diagnosis of blastic NK-cell lymphoma was made and theskin lesions regressed after treatment with L-asparaginase-based chemotherapy (L-asparaginase, cyclophosphamide, etoposide). The skin lesions regressed after two sessions of chemotherapy.