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爱泼斯坦-巴尔病毒在X连锁淋巴增殖性疾病中诱导噬血细胞性淋巴组织细胞增生症。

Epstein-Barr virus induced hemophagocytic lymphohistiocytosis in X-linked lymphoproliferative disease.

作者信息

Sankararaman Senthilkumar, Riel-Romero Rosario Maria, Jeroudi Majed, Gonzalez-Toledo Eduardo

机构信息

Department of Pediatrics, Louisiana State University Health Sciences Center, 1501 Kings Highway, Shreveport, LA, USA.

Department of Pediatrics and Neurology, Louisiana State University Health Sciences Center, 1501 Kings Highway, Shreveport, LA, USA.

出版信息

J Neurosci Rural Pract. 2014 Apr;5(2):171-4. doi: 10.4103/0976-3147.131669.

DOI:10.4103/0976-3147.131669
PMID:24966560
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4064187/
Abstract

X-linked lymphoproliferative disease (XLP) is a rare, often fatal genetic disorder characterized by extreme vulnerability to Epstein-Barr virus (EBV). EBV-induced hemophagocytic lymphohistiocytosis (HLH) is a known presentation in XLP. In EBV-induced HLH in XLP, the brain imaging findings in the acute phase include a non specific pattern. In this report, we highlight the magnetic resonance imaging and magnetic resonance spectroscopy findings in a child with EBV induced HLH in XLP.

摘要

X连锁淋巴细胞增生性疾病(XLP)是一种罕见的、通常致命的遗传性疾病,其特征是极易感染爱泼斯坦-巴尔病毒(EBV)。EBV诱导的噬血细胞性淋巴组织细胞增生症(HLH)是XLP的一种已知表现形式。在XLP中EBV诱导的HLH的急性期,脑部影像学表现为非特异性模式。在本报告中,我们重点介绍了一名患有XLP中EBV诱导HLH的儿童的磁共振成像和磁共振波谱结果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/0250c47c937a/JNRP-5-171-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/09352c94375c/JNRP-5-171-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/33e722acce84/JNRP-5-171-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/0250c47c937a/JNRP-5-171-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/09352c94375c/JNRP-5-171-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/33e722acce84/JNRP-5-171-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc08/4064187/0250c47c937a/JNRP-5-171-g003.jpg

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Commentary.评论
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Neuroradiologic findings and follow-up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement.遗传性噬血细胞性淋巴组织细胞增生症伴中枢神经系统受累的神经放射学表现及磁共振成像随访。
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X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease.
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Blood. 2011 Jan 6;117(1):53-62. doi: 10.1182/blood-2010-06-284935. Epub 2010 Oct 6.
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