Intravascular NK-cell lymphoma: a case report and review of the literature.

Intravascular NK-cell lymphoma: a case report and review of the literature.
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DOI:
10.1186/s13000-015-0336-7
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发表时间:
2015-07-01
影响因子:
2.6
通讯作者:
Liu T
Liu T
中科院分区:
医学4区
文献类型:
--
作者:
Bi Y;Huo Z;Liang Z;Meng Y;Jia C;Shi X;Song L;Luo Y;Ling Q;Liu T

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血管内NK细胞淋巴瘤(IVNKL)是一种非常罕见的非霍奇金淋巴瘤的变种。据我们所知,在英国文献中只有少数病例报道。在这里,一个29岁的男性与IVNKL涉及皮肤的躯干和四肢和肝脏的情况下。本文就此疾病的临床及病理特徴作一全面的文献回顾。在我们的病例中,大的肿瘤性淋巴样细胞局限于小血管的管腔内,表现出真正的NK细胞的表型。其形态学、免疫表型、EBER阳性及NK细胞来源与其他IVNKL病例相似。此外,包括我们在内的一些病例预后不良,因为涉及多个系统或重要器官。总之,我们报告一例罕见的血管内淋巴瘤的NK细胞谱系,显示临床和病理特征,并与其他鉴别诊断进行比较。重要的是要认识到这种罕见的实体,以作出适当的诊断,并实现更好地了解有关的治疗和预后。
Intravascular NK-cell lymphoma (IVNKL) is an extremely rare variant of non-Hodgkin lymphoma. To our knowledge, there are only a few cases reported in the English literature. Here, a case of a 29-year-old male with IVNKL involving the skin of the trunk and 4 extremities and liver is presented. A comprehensive literature review is undertaken to summarize the clinical and pathological features of this disorder. In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell. The morphology and immunophenotype, positivity of EBER and NK-cell origin are similar to other IVNKL cases. In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved. In summary, we report a case of an unusual intravascular lymphoma of NK-cell lineage that displays both clinical and pathological features and compare it with other differential diagnoses. It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.
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