中华皮肤科杂志 ›› 2011, Vol. 44 ›› Issue (8): 574-577.

• 论著 • 上一篇    下一篇

以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤一例

马寒1,邵春奎2,3,梁琼3,苏祖兰3,刘加军2,4,陆春5   

  1. 1. 广州市中山大学附属第三医院皮肤科
    2.
    3. 广州 中山大学附属第三医院病理科
    4. 广州 中山大学附属第三医院血液科
    5. 广州中山大学附属第三医院
  • 收稿日期:2010-10-20 修回日期:2010-12-22 出版日期:2011-08-15 发布日期:2011-07-26
  • 通讯作者: 陆春 E-mail:luliyuan@tom.com

Intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions: a case report

  • Received:2010-10-20 Revised:2010-12-22 Online:2011-08-15 Published:2011-07-26

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摘要：

患者男，51岁，上腹部胀痛4个月，颈项、躯干、双大腿结节、斑块1个月。当地多家医院行胃肠道相关检查，并予对症处理，治疗效果不佳。腹部皮肤结节组织病理检查：真皮下层和皮下脂肪小叶内大量增生的小血管腔内填塞中等偏大异形淋巴样细胞；免疫组化标记：血管腔内异形细胞CD2（+++）、CD4（-）、CD8（-）、CD3ε胞质（++）、CD99（++）、CD43（+++）、CD56（+++）、细胞毒颗粒相关蛋白（TIA-1）（++）、穿孔素（++）、EBER（+++）、CD20（-）、CD79a（-）、CD30（-）、细胞角蛋白（-）、S100（-）、CD68（-）、血管内皮细胞CD31（+）、CD34（+）。诊断：以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤。给予环磷酰胺、长春新碱、柔红霉素、地塞米松 + 依托泊苷方案化疗，病情得到迅速控制，目前仍在随访中。

关键词: NK/T细胞

Abstract:

A 51-year-old man presented with a 4-month history of upper abdominal distending pain and 1-month history of cutaneous nodules and plaques on the neck, trunk and bilateral thighs. The patient underwent many gastrointestinal tract examinations in several local hospitals, and symptomatic treatment did not work. The biopsy of nodules on the abdomen revealed medium- to large-sized atypical lymphoid cells within numerous small vessels in lower dermis and subcutaneous fat tissue. Additionally, the atypical cells were present exclusively within vascular lumina. Immunohistochemical labeling showed the reactivity of neoplastic cells to CD2, CD99, CD3ε, CD43, CD56, Epstein-Barr virus-encoded small nuclear RNAs （EBER）, and cytotoxic proteins such as T-cell intracellular antigen-1 （TIA-1） and perforin, but not to CD4, CD8, CD20, CD79a, CD30, cytokeratin （CK）, S100, or CD68. The endothelial cells lining the involved vessels exhibited the reactivity to CD31 and CD34. Based on the above findings, the patient was diagnosed with intravascular NK/T-cell lymphoma firstly manifesting as gastrointestinal tract symptom and complicated by skin lesions. Following combined chemotherapy with cyclophosphamide, daunorubicin, vincristine, prednisone and etoposide, the patient experienced a quick and satisfactory recovery and the follow-up still continued.

Key words: NK/T-cell