鲁索替尼治疗后t细胞-爱泼斯坦-巴尔病毒-噬血细胞淋巴组织细胞增多症1例

IF 4.6 2区 医学 Q2 IMMUNOLOGY Clinical & Translational Immunology Pub Date : 2023-07-25 DOI:10.1002/cti2.1459
Syed Ali, Sharon Choo, Laine Hosking, Anthony Smith, Tiffany Hughes
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引用次数: 1

摘要

目的eb病毒(EBV)是继发性噬血细胞淋巴组织细胞病(HLH)的常见病因。虽然B细胞是eb病毒的宿主,但在这种疾病中,T细胞和NK细胞内的感染很难治疗。方法一名19岁女性,以克罗恩病为背景,有6周的鼻塞症状。检查时,她有发热和心动过速,伴有轻度扁桃体肿大和脾肿大。检测到新的三岁细胞减少和肝酶升高,随后全血PCR证实急性EBV。血清铁蛋白、甘油三酯和可溶性CD25升高,纤维蛋白原低,骨髓中存在噬血细胞现象,进一步支持ebv相关HLH的诊断。结果给予皮质类固醇、IVIG和利妥昔单抗治疗,由于持续发热,随后加用阿那单抗治疗。然后在EBER Flow-FISH实验中证实了EBV感染在CD8+ T细胞内。开始使用Ruxolitinib,第5天患者退烧,其他HLH参数有所改善。她在住院39天后出院。迄今为止,尽管在HLH恢复期发生了COVID-19感染,但她的HLH仍处于缓解期。结论EBV病毒血症需要适当的治疗来控制EBV相关的HLH,因为美罗昔单抗可能不足,皮质类固醇耐药可导致CD8+ T细胞持续感染EBV。这种实体被称为t细胞- ebv - hlh。Ruxolitinib在这种特殊情况下是一种新的治疗策略,具有几个优点,包括抑制皮质类固醇耐药,促进ebv感染的T细胞凋亡。
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A case of T-cell-Epstein–Barr virus-haemophagocytic lymphohistiocytosis and sustained remission following ruxolitinib therapy
Epstein–Barr virus (EBV) is a common cause of secondary haemophagocytic lymphohistiocytosis (HLH). While B cells are reservoirs for EBV, infection within T cells and NK cells in this disease can be difficult to treat.
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来源期刊
Clinical & Translational Immunology
Clinical & Translational Immunology Medicine-Immunology and Allergy
CiteScore
12.00
自引率
1.70%
发文量
77
审稿时长
13 weeks
期刊介绍: Clinical & Translational Immunology is an open access, fully peer-reviewed journal devoted to publishing cutting-edge advances in biomedical research for scientists and physicians. The Journal covers fields including cancer biology, cardiovascular research, gene therapy, immunology, vaccine development and disease pathogenesis and therapy at the earliest phases of investigation.
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