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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Treosulfan-based conditioning in DOCK8 deficiency: Complete lympho-hematopoietic reconstitution with minimal toxicity
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Treosulfan-based conditioning in DOCK8 deficiency: Complete lympho-hematopoietic reconstitution with minimal toxicity

机译：基于Teck8缺陷的基于Treosulfan的调理：完全淋巴 - 造血重构，毒性最小

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摘要

Autosomal-recessive Hyper IgE syndrome (HIES) has been attributed to mutations in the dedicator of cytokinesis 8 (DOCK8) gene causing combined immunodeficiency [1], HIES patients suffer from recurrent sino-pulmonary and skin infections, atopy, viral infections and chronic muco-cutaneous candidiasis. In addition to infectious complications a higher propensity for developing malignancies contributes to HIES-associated morbidity and mortality [2]; till now, however, only few case reports have addressed allogeneic hematopoietic stem cell transplantation (HSCT) in DOCK8 deficient patients [3-7].
机译：常染色体 - 隐性超IgE综合征（HIES）已归因于细胞因子8（Dock8）基因的突变导致综合免疫缺陷[1]，HIES患者患有复发性中肺和皮肤感染，Atacty，病毒感染和慢性粘膜 - 念珠菌病。 除了传染性并发症外，对恶性肿瘤的更高倾向有助于HIE相关的发病率和死亡率[2]; 然而，直到现在，只有很少的病例报告已经解决了在Dock8缺陷患者中的同种异体造血干细胞移植（HSCT）[3-7]。

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    GhoshS.; SchusterF.R.; FuchsI.; LawsH.-J.; BorkhardtA.; MeiselR.;

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    Department of Pediatric Oncology Hematology and Clinical Immunology Medical Faculty Center of;

    Department of Pediatric Oncology Hematology and Clinical Immunology Medical Faculty Center of;

    Centre of Chronic Immunodeficiency (CCI) University Medical Center Freiburg and University of;

    Department of Pediatric Oncology Hematology and Clinical Immunology Medical Faculty Center of;

    Department of Pediatric Oncology Hematology and Clinical Immunology Medical Faculty Center of;

    Department of Pediatric Oncology Hematology and Clinical Immunology Medical Faculty Center of;

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  • 正文语种 eng
  • 中图分类 医学免疫学;
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