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儿童免疫性血小板减少性紫癜发病机制及治疗中的免疫学方面

Immunologic aspects in the pathogenesis and treatment of immune thrombocytopenic purpura in children.

作者信息

Imbach P A, Kühne T, Holländer G

机构信息

Children's Hospital of Basel, Switzerland.

出版信息

Curr Opin Pediatr. 1997 Feb;9(1):35-40. doi: 10.1097/00008480-199702000-00009.

Abstract

Immune thrombocytopenic purpura (ITP) is either a transient or a chronic bleeding disorder characterized by an unbalanced immune response due to inflammation (ie, infection, autoimmunity). There is new evidence arguing for an immune-mediated mechanism operative in ITP-increased HLA-DR expression, defects in cellular and humoral immunity, and specific autoantibody production. Moreover, different forms of ITP may be associated with specific alterations in the immune response. New immunosupportive modalities emphasize biologic interventions as a therapeutic strategy for autoimmune disorders. Practical guidelines for the diagnosis and treatment of ITP were recently established by the American Society of Hematology. These recommendations will substantially influence patient care and future research.

摘要

免疫性血小板减少性紫癜(ITP)是一种短暂性或慢性出血性疾病,其特征为因炎症(如感染、自身免疫)导致免疫反应失衡。有新证据支持ITP中存在免疫介导机制——HLA-DR表达增加、细胞免疫和体液免疫缺陷以及特异性自身抗体产生。此外,不同形式的ITP可能与免疫反应的特定改变有关。新的免疫支持模式强调生物干预作为自身免疫性疾病的治疗策略。美国血液学会最近制定了ITP诊断和治疗的实用指南。这些建议将对患者护理和未来研究产生重大影响。

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