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学科主题: 临床医学
题名:
Primary Central Nervous System Extranodal NK/T Cell Lymphoma, Nasal Type, with Antecedent Hemophagocytic Syndrome in a Child
作者: 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
发表日期: 2014-11-01
DOI: 10.2350/14-02-1441-CR.1
卷: 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
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/67162
Appears in Collections:北京大学深圳医院_病理科_期刊论文

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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

Recommended Citation:
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.
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