目标：据我们所知，自2003年以来，已有11例报道血管内自然杀伤（NK）细胞淋巴瘤（IVNKL）。这里我们描述的是第12种情况。
方法：采用H&E和Envision免疫组织化学染色及原位杂交结合文献复习对本病进行研究。
结果：迄今为止报告的病例中有一半来自中国和台北。IVNKL的临床表现为四肢和躯干红斑，尽管患者的病情各不相同。一年生存率约为40%。
结论：IVNKL应与结外NK/T细胞淋巴瘤（鼻型）和侵袭性NK细胞白血病区分开来。这三种疾病的表型相似，均与EB病毒感染有关。然而,发病机制的异同有待进一步研究。特别是,IVNKL很不寻常。IVNKL的治疗是困难的，预后较差。目前,IVNKL不包括在世界卫生组织分类亚型中，已被分类为NK/T细胞淋巴瘤（鼻型）。然而,鉴于这种疾病的独特特征，我们建议诊断是独立的，因为这将有助于对这种疾病的进一步研究。
OBJECTIVE: To our knowledge, since 2003, there have been 11 reported cases of intravascular natural killer (NK)-cell lymphoma (IVNKL). Herein we describe the 12th
case.
METHODS: H&E and Envision immunohistochemical stains as well as in situ hybridization were used to study this disease in combination with review of the literature.
RESULTS: Half of the cases reported to date are from China and Taipei. The clinical manifestation of IVNKL is erythema in the limbs and trunk, although patients\' conditions have varied notably from each other. One-year survival rate is about 40%.
CONCLUSIONS: IVNKL should be distinguished from extranodal NK/T-cell lymphoma (nasal type) and aggressive NK-cell leukemia. These three diseases have a similar phenotype and are all related to Epstein-Barr virus infection. However, the pathogenesis of similarities and differences needs further study. In particular, IVNKL is quite unusual. The treatment of IVNKL is difficult, and the prognosis is poor. Currently, IVNKL is not included in the World Health Organization classification subtypes and has been classified into NK/T-cell lymphoma (nasal type). However, in view of the unique characteristics of this disease, we propose that the diagnosis be independent, since this will facilitate further study of this disease.
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