Partial and Transient Clinical Response to Omalizumab in IL-21-Induced Low STAT3-Phosphorylation on Hyper-IgE Syndrome

Pub Date : 2019-07-04 DOI:10.1155/2019/6357256
C. D. Alonso-Bello, M. Jiménez-Martínez, M. E. Vargas-Camaño, S. Hierro-Orozco, M. Ynga-Durand, L. Berrón-Ruiz, J. C. Alcántara-Montiel, L. Santos‐Argumedo, Diana Andrea Herrera-Sánchez, Fernando Lozano-Patiño, María Isabel Castrejón-Vázquez
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引用次数: 10

Abstract

Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency characterized by elevated levels of immunoglobulin E (IgE), eczematous dermatitis, cold abscesses, and recurrent infections of the lung and skin caused by Staphylococcus aureus. The dominant form is characterized by nonimmunologic features including skeletal, connective tissue, and pulmonary abnormalities in addition to recurrent infections and eczema. Omalizumab is a humanized recombinant monoclonal antibody against IgE. Several studies reported clinical improvement with omalizumab in patients with severe atopic eczema with high serum IgE level. We present the case of a 37-year-old male with HIES and cutaneous manifestations, treated with humanized recombinant monoclonal antibodies efalizumab and omalizumab. After therapy for 4 years, we observed diminished eczema and serum IgE levels.
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Omalizumab对il -21诱导的高ige综合征低stat3磷酸化的部分和短暂临床反应
高IgE综合征(HIES)是一种罕见的原发性免疫缺陷,其特征是免疫球蛋白E (IgE)水平升高,湿疹性皮炎,冷脓肿以及由金黄色葡萄球菌引起的肺部和皮肤复发性感染。主要形式的特点是非免疫特征,包括骨骼、结缔组织和肺部异常,以及复发性感染和湿疹。Omalizumab是一种人源化重组抗IgE单克隆抗体。一些研究报道了奥玛珠单抗对血清IgE水平高的严重特应性湿疹患者的临床改善。我们报告一例37岁男性HIES和皮肤表现,治疗人源化重组单克隆抗体依法利珠单抗和奥玛利珠单抗。治疗4年后,我们观察到湿疹和血清IgE水平降低。
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