全血细胞减少症
医学
骨髓衰竭
再生障碍性贫血
免疫抑制
免疫学
骨髓
环磷酰胺
干细胞
免疫系统
病理生理学
造血
化疗
内科学
生物
遗传学
作者
Robert A. Brodsky,Richard J. Jones
出处
期刊:The Lancet
[Elsevier]
日期:2005-05-01
卷期号:365 (9471): 1647-1656
被引量:219
标识
DOI:10.1016/s0140-6736(05)66515-4
摘要
Aplastic anaemia is a rare haemopoietic stem-cell disorder that results in pancytopenia and hypocellular bone marrow. Although most cases are acquired, there are unusual inherited forms. The pathophysiology of acquired aplastic anaemia is immune mediated in most cases; autoreactive lymphocytes mediate the destruction of haemopoietic stem cells. Environmental exposures, such as to drugs, viruses, and toxins, are thought to trigger the aberrant immune response in some patients, but most cases are classified as idiopathic. Similarly to other autoimmune diseases, aplastic anaemia has a varied clinical course; some patients have mild symptoms that necessitate little or no therapy, whereas others present with life-threatening pancytopenia representing a medical emergency. Paroxysmal nocturnal haemoglobinuria and myelodysplastic syndrome commonly arise in patients with aplastic anaemia, showing a pathophysiological link between these disorders. Acquired aplastic anaemia can be effectively treated by allogeneic bone-marrow transplantation, immunosuppression (generally antithymocyte globulin and ciclosporin), and high-dose cyclophosphamide.
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