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Diagn Pathol. 2015 Jul 1;10:84. doi: 10.1186/s13000-015-0336-7.

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

Author information

1
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Biyeye81@126.com.
2
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Huozhen1976@126.com.
3
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. LiangZY@pumch.cn.
4
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. MengYX@pumch.cn.
5
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. David_jia0814@163.com.
6
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Shixiaohua3762@hotmail.com.
7
Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. sallysonglan@sina.com.
8
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. LuoYF@pumch.cn.
9
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. LingQ@pumch.cn.
10
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Liuth_pumch192911@yahoo.com.

Abstract

BACKGROUND:

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.

FINDINGS:

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.

CONCLUSION:

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.

PMID:
26126576
PMCID:
PMC4488042
DOI:
10.1186/s13000-015-0336-7
[Indexed for MEDLINE]
Free PMC Article

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