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尽管存在严重的并发感染,仍用emapalumab 治疗难治性噬血细胞性淋巴组织细胞增生症。

Treatment of refractory hemophagocytic lymphohistiocytosis with emapalumab despite severe concurrent infections.

机构信息

Cancer and Blood Diseases Institute and.

Division of Immunobiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

出版信息

Blood Adv. 2019 Jan 8;3(1):47-50. doi: 10.1182/bloodadvances.2018025858.


DOI:10.1182/bloodadvances.2018025858
PMID:30617216
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6325304/
Abstract

Neutralization of IFN-γ with emapalumab can reverse severe, refractory hemophagocytic lymphohistiocytosis. Neutralizing IFN-γ did not impair control of multiple viral and other infections in a severely ill patient.

摘要

使用emapalumab 中和 IFN-γ 可逆转严重、难治性噬血细胞性淋巴组织细胞增生症。中和 IFN-γ 并未损害严重疾病患者对多种病毒和其他感染的控制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70e1/6325304/cfa3e61a3a51/advances025858absf1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70e1/6325304/cfa3e61a3a51/advances025858absf1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/70e1/6325304/cfa3e61a3a51/advances025858absf1.jpg

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Treatment of refractory hemophagocytic lymphohistiocytosis with emapalumab despite severe concurrent infections.

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[9]
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本文引用的文献

[1]
Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study.

Blood. 2017-12-21

[2]
Neutralization of IFN-γ reverts clinical and laboratory features in a mouse model of macrophage activation syndrome.

J Allergy Clin Immunol. 2017-8-12

[3]
Salvage therapy for refractory hemophagocytic lymphohistiocytosis: A review of the published experience.

Pediatr Blood Cancer. 2016-10-27

[4]
Use of a mouse model to identify a blood biomarker for IFNγ activity in pediatric secondary hemophagocytic lymphohistiocytosis.

Transl Res. 2017-2

[5]
Elevated circulating levels of interferon-γ and interferon-γ-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

Ann Rheum Dis. 2016-6-13

[6]
Pathogenesis of Hemophagocytic Lymphohistiocytosis.

Hematol Oncol Clin North Am. 2015-10

[7]
Cytokines in systemic juvenile idiopathic arthritis and haemophagocytic lymphohistiocytosis: tipping the balance between interleukin-18 and interferon-γ.

Rheumatology (Oxford). 2015-3-12

[8]
Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab.

Pediatr Blood Cancer. 2012-4-22

[9]
Diagnostic accuracy of a specific cytokine pattern in hemophagocytic lymphohistiocytosis in children.

J Pediatr. 2012-1-9

[10]
Expression of type I interferon by splenic macrophages suppresses adaptive immunity during sepsis.

EMBO J. 2011-10-28

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