当前位置: X-MOL 学术Curr. Pharm. Biotechnol. › 论文详情
Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)
Idiopathic Aplastic Anemia in Children and Adults: Diagnosis, Treatments, and Management - A Review
Current Pharmaceutical Biotechnology ( IF 2.2 ) Pub Date : 2020-10-31 , DOI: 10.2174/1389201021666191210141426
Fatmah S Alqahtany 1
Affiliation  

Aplastic Anemia (AA) is also known as idiopathic aplastic anemia (IAA) and the production of new blood cells ceases in AA, which leads to an abnormal hematological syndrome such as pancytopenia and suppression of hypo-cellular bone marrow. The pathophysiology of AA in most cases is immune-mediated and is stimulated by type 1 cytotoxic T cells. Reliable early diagnostic tests of IAA are not yet available, therefore most of the cases are identified in advanced stages. Recently, abnormal immune response and hematopoietic cell deficiencies are defined genetically, such as in target cells of telomere repair gene mutations and by the dysregulation of T-cell activation pathways. Importantly, anti-thymocyte globulins and cyclosporine-associated immunosuppression are successful treatments for restoring blood cell production in most of the cases, however, clonal hematologic diseases remain challenging. In the current review, we have discussed the common practices in the treatment, pathophysiology, diagnosis, and management of AA.



中文翻译:

儿童和成人特发性再生障碍性贫血:诊断,治疗和管理-审查。

再生障碍性贫血(AA)也称为特发性再生障碍性贫血(IAA),新血细胞的产生在AA中停止,这会导致异常的血液学综合征,例如全血细胞减少症和抑制低细胞性骨髓。在大多数情况下,AA的病理生理是免疫介导的,并由1型细胞毒性T细胞刺激。目前尚无法获得可靠的IAA早期诊断测试,因此大多数病例已被确定为晚期。最近,异常的免疫反应和造血细胞缺乏症在遗传学上得到了定义,例如在端粒修复基因突变的靶细胞中以及通过T细胞活化途径的失调。重要的是,在大多数情况下,抗胸腺细胞球蛋白和环孢素相关的免疫抑制是恢复血细胞生成的成功方法,但是,克隆血液学疾病仍然具有挑战性。在本综述中,我们讨论了AA的治疗,病理生理,诊断和管理的常见做法。

更新日期:2020-12-01
down
wechat
bug