嗜酸性粒细胞增多综合征的诊断和治疗方法

D. Leković
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摘要

嗜酸性粒细胞增多综合征定义为外周血嗜酸性粒细胞计数?1.5 ?109/L与组织或器官损伤有关。嗜酸性粒细胞疾病是一组病理生理、临床表现和预后均不相同的病理状况。疾病的预后是基于确定嗜酸性粒细胞增多症的亚型和机制。基于症状和体征的诊断来评估器官损害的程度是很重要的。在排除嗜酸性粒细胞增多症的继发原因后,2016年,世界卫生组织批准了一项针对疾病亚型的分子分类方案的评估,称为克隆性或原发性嗜酸性粒细胞增多症。原发性嗜酸性粒细胞增多症的诊断评估依赖于血液和骨髓的形态学检查、标准细胞遗传学、荧光原位杂交、流式免疫表型和t细胞克隆性评估的结合,以检测急性或慢性骨髓/淋巴肿瘤的组织病理学或克隆证据。治疗的目的是减少嗜酸性粒细胞介导的器官损伤。根据嗜酸性粒细胞增多症的病因,治疗意义从观察和等待?实现同种异体造血干细胞移植。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hypereosinophilic syndrome - diagnostic and treatment approach
Hypereosinophilic syndrome is defined as a peripheral blood eosinophil count ? 1.5 ? 109/L associated with tissue or organ damage. Eosinophilic disorders represent a group of pathological conditions with heterogeneous pathophysiology, clinical presentation and prognosis. The disease prognosis is based on identifying the subtype and mechanism of eosinophilia. It is important to assess the degree of organ damage based on diagnostics that is directed upon symptoms and signs. After exclusion of secondary causes of eosinophilia, in 2016, the World Health Organization endorsed an assessment towards a molecular classification scheme of disease subtypes named clonal or primary eosinophilias. Diagnostic evaluation of primary eosinophilia relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescence in situ hybridization, flow immunophenotyping, and a T-cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid/lymphoid neoplasm. The goal of the therapy is to reduce eosinophil-mediated organ damage. Depending of cause of eosinophilia therapeutic implications range from a ?watch and wait? to the implementation of allogeneic hematopoietic stem cell transplantation.
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