[伴有严重后果的爱泼斯坦-巴尔病毒感染。爱泼斯坦-巴尔病毒、噬血细胞性淋巴组织细胞增生症与唐氏综合征相关的霍奇金淋巴瘤]
[Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome].
作者信息
Kuitert Pieter C J, Abbink Floor C H, Broers Chantal J M, van der Valk Paul, van Furth A Marceline, van der Kuip Martijn
机构信息
VU medisch centrum, afd. Kindergeneeskunde, Amsterdam.
出版信息
Ned Tijdschr Geneeskd. 2014;158:A7608.
BACKGROUND
Over 90% of the population is infected with the Epstein-Barr virus (EBV). Following primary infection, the virus remains latent in B-lymphocytes. In isolated cases, especially in immunocompromised patients, the Epstein-Barr virus can result in a chronic active infection (CAEBV).
CASE DESCRIPTION
We describe an 11-year-old boy with Down syndrome who was admitted because of fever of unknown origin during several periods. Serological findings (high VCA-IgG and absent EBNA-IgG) were suggestive of CAEBV, which was confirmed by the high circulating EBV viral load. During admission the clinical picture worsened and our patient developed pancytopenia, which led us to diagnose concurrent haemophagocytic lymphohistiocytosis and Hodgkin lymphoma.
CONCLUSION
CAEBV is the result of deficiency in cellular immunity, which in this patient could possibly be attributed to deficiencies in the immune system associated with Down syndrome. CAEBV is difficult to treat, and it can come with life-threatening complications such as haemophagocytic lymphohistiocytosis.
背景
超过90%的人口感染过爱泼斯坦-巴尔病毒（EBV）。初次感染后，该病毒会潜伏在B淋巴细胞中。在个别情况下，尤其是免疫功能低下的患者，爱泼斯坦-巴尔病毒可导致慢性活动性感染（CAEBV）。
病例描述
我们描述了一名患有唐氏综合征的11岁男孩，他因多次不明原因发热入院。血清学检查结果（高VCA-IgG和EBNA-IgG阴性）提示CAEBV，高循环EBV病毒载量证实了这一点。住院期间临床症状恶化，我们的患者出现全血细胞减少，这使我们诊断为并发噬血细胞性淋巴组织细胞增生症和霍奇金淋巴瘤。
结论
CAEBV是细胞免疫缺陷的结果，在该患者中可能归因于与唐氏综合征相关的免疫系统缺陷。CAEBV难以治疗，并且可能伴有噬血细胞性淋巴组织细胞增生症等危及生命的并发症。
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