Nasal-type Extranodal Natural Killer/T-cell Lymphoma Present with Chronic Leg Ulcer-Case Presentation and Literature Review

以慢性潰瘍傷口呈現之鼻腔型淋巴腺外NK／T細胞淋巴瘤－病歷報告及文獻回顧

黃柏誠(Po-Cheng Huang) ； 謝榮賢(Jung-Hsien Hsieh) ； 陳明庭(Ming-Ting Chen)

《臺灣整形外科醫學會雜誌》 22卷2期 (2013/06) Pp. 143-151

摘要

背景：鼻腔型淋巴腺外NK/T細胞淋巴瘤是一種罕見的人類皰疹病毒第四型相關原發性細胞毒性淋巴瘤。相較於西方國家，亞洲地區的發生頻率高於四倍。NK/T細胞淋巴瘤病灶中以鼻腔及鼻咽處佔首位，皮膚次之，其他地方包含腸胃系統，軟組織，腦，肝，脾，骨髓及睪丸。目的及目標：報告一罕見鼻腔型淋巴腺外NK/T細胞淋巴瘤病歷及文獻回顧。材料及方法：病人最先是以慢性左腳潰瘍性傷口表現，經清創後病理報告呈現鼻腔型淋巴腺外NK/T 細胞淋巴瘤，其CD56及CD30呈陽性，CD3及EBV原位染色為局部陽性，CD20、CD4、CD8及ALK幾乎為陰性反應，升高之anti-β2 glycoprotein 1疑似與腫瘤伴生徵候群相關。結果：經L-COP化學治療，皮膚病灶改善且表皮重新生長，經切片檢查病無惡性淋巴瘤存在。結論：鼻腔型淋巴腺外NK/T細胞淋巴瘤預後非常不好。若病人存在一個未癒合或擴大的潰瘍，多次的切片檢查及臨床病理的配合是正確診斷的重要步驟，皮膚淋巴瘤也必須視為鑑別診斷之一。

並列摘要

Background:Nasal-type extranodal natural killer (NK)/T-cell lymphoma is a rare type of cytotoxic primary cutaneous lymphoma associated with the Epstein–Barr virus (EBV). This disease is more common in Asia, appearing four times more frequently compared to Western countries. The skin is the second most common site for NK/T-cell lymphomas, after the nasal cavity and nasopharynx. Other sites include the gastrointestinal system, soft tissue, brain, liver, spleen, bone marrow and testes.Aim and objective:Present a rare case of nasal-type extranodal NK/T-cell lymphoma, the course of treatment, and a review of the literature.Materials and methods:We present a case with an initial presentation of chronic ulceration of the left leg. After receiving skin debridement, the pathology showed nasal-type extranodal natural killer (NK)/T-cell lymphoma, with positive results for CD56, CD30, focal positive for CD3 and EBV in situ hybridization, while mostly negative for CD20, CD4, CD8 and ALK. Elevated anti-β2 glycoprotein 1 levels resulted in suspected paraneoplastic syndrome secondary to lymphoma.Result:After L-COP regimen therapy, the skin lesion showed improvement and reepithelialization. A repeat biopsy showed no malignant lymphoma.Conclusion:Nasal-type extranodal natural killer/T-cell lymphoma has an extremely poor prognosis. In patients with nonhealing or extensive leg ulcers, repeat biopsies and close clinicopathologic correlation are essential for the establishment of a correct diagnosis, and cutaneous lymphoma should be considered as a differential diagnosis.

並列關鍵字

extranodal natural killer (NK)/T-cell lymphoma ； CD56 ； anti-β2 glycoprotein 1