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学科主题临床医学
Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child
Jiang, Xianping1; Yin, Weihua2; Song, Jianming1; Chen, Xiaowen3; Zhao, Cailei4; Wen, Feiqiu3
关键词central nervous system children hemophagocytic syndrome NK/T cell lymphoma TCR gene rearrangement
刊名PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
2014-11-01
DOI10.2350/14-02-1441-CR.1
17期:6页:482-486
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Pathology ; Pediatrics
研究领域[WOS]Pathology ; Pediatrics
关键词[WOS]EPSTEIN-BARR-VIRUS ; GENE REARRANGEMENTS ; CLINICAL-FEATURES ; LYMPHOHISTIOCYTOSIS ; IMMUNOGLOBULIN ; BRAIN
英文摘要

Primary central nervous system (CNS) extranodal natural killer (NK)/T-cell lymphoma, nasal type (NKTCL), is an exceedingly uncommon entity. Here, we present a case of CNS NKTCL that manifested initially as hemophagocytic syndrome 4 months earlier in a 13-year-old girl. Histological examination revealed the cerebellum mass was composed of large-sized and atypical tumor cells, with an angiocentric and angiodestructive growth pattern and prominent necrosis. The tumor cells exhibited marked pleomorphism with conspicuous nucleoli and prominent mitotic activity. Immunohistochemical staining showed the tumor cells were positive for CD45, CD2, CD3e, CD30, CD43, CD56, and granzyme B. Epstein-Barr virus-encoded ribonucleic acid was expressed in almost all of the nuclei of the lymphoma cells. The T-cell receptor gamma chain gene rearrangement study showed no evidence of a clonal rearrangement. The patient was treated with etoposide and dexamethasone and died a few days after the operation. As far as we know, this case is the 1st pediatric and female patient of primary CNS NKTCL with antecedent hemophagocytic syndrome, which highlights the clinical data and is helpful for the diagnosis of this tumor.

语种英语
WOS记录号WOS:000348609300012
引用统计
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/67162
专题北京大学深圳医院_病理科
作者单位1.Shenzhen Childrens Hosp, Dept Pathol, Shenzhen 518026, Peoples R China
2.Peking Univ, Shenzhen Hosp, Dept Pathol, Shenzhen 518036, Peoples R China
3.Shenzhen Childrens Hosp, Dept Hematol & Oncol, Shenzhen 518026, Peoples R China
4.Shenzhen Childrens Hosp, Dept Radiol, Shenzhen 518026, Peoples R China
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Jiang, Xianping,Yin, Weihua,Song, Jianming,et al. Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child[J]. PEDIATRIC AND DEVELOPMENTAL PATHOLOGY,2014,17(6):482-486.
APA Jiang, Xianping,Yin, Weihua,Song, Jianming,Chen, Xiaowen,Zhao, Cailei,&Wen, Feiqiu.(2014).Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child.PEDIATRIC AND DEVELOPMENTAL PATHOLOGY,17(6),482-486.
MLA Jiang, Xianping,et al."Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child".PEDIATRIC AND DEVELOPMENTAL PATHOLOGY 17.6(2014):482-486.
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