无细胞性贫血是什么?
Robert A Brodsky1, Richard J Jones
1Johns Hopkins University School of Medicine, Division of Hematology, and Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD, USA. brodsro@jhmi.edu
Lancet (London, England)
|May 12, 2005
概括
无质性贫血是一种罕见的干细胞疾病,导致泛细胞衰竭. 血液造血干细胞的免疫媒介破坏是主要原因,有效的治疗方法包括移植和免疫抑制.
科学领域:
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
- 遗传学 是一个遗传学.
背景情况:
- 无质性贫血是一种罕见的,危及血液造血干细胞的生命的疾病,其特征是泛细胞衰竭和低细胞骨髓.
- 虽然大多数病例是获得的和免疫介导的,但存在罕见的遗传形式.
- 病理生理学涉及自身反应性淋巴细胞破坏造血干细胞,可能由环境因素引发.
研究的目的:
- 总结一下无塑性贫血的病理生理学,临床过程和治疗方法.
- 为了突出表现出无塑性贫血,阳性夜间血红蛋白尿和骨髓发育综合征之间的联系.
主要方法:
- 关于无塑性贫血病理生理学和治疗的文献综述.
- 对参与造血干细胞破坏的免疫媒介机制的分析.
- 审查已建立和新兴的治疗策略.
主要成果:
- 获得的无形性贫血主要是免疫介导的,最常见的是异常病例.
- 疾病的过程是可变的,从轻度到危及生命的泛细胞衰竭.
- 性夜间血红蛋白尿和骨髓质疏松综合征是常见的后果,表明共享的病理生理路径.
结论:
- 获得的无形性贫血的有效治疗方法包括全基骨髓移植,免疫抑制 (抗甲基球蛋白和环素) 和高剂量环胺.
- 了解免疫基础对于管理这种罕见的血液疾病至关重要.
相关概念视频
Alterations in Blood Pressure
Alterations in blood pressure, such as hypertension (high blood pressure) and hypotension (low blood pressure), significantly affect human health. Understanding these conditions' classifications, causes, and symptoms is essential for effective management and treatment.
Hypertension (High blood pressure)
Hypertension occurs when blood pressure readings consistently exceed the normal range. It is diagnosed when systolic blood pressure (the top number, indicating pressure while the heart beats)...
Hypertension (High blood pressure)
Hypertension occurs when blood pressure readings consistently exceed the normal range. It is diagnosed when systolic blood pressure (the top number, indicating pressure while the heart beats)...
Disorders of Hemostasis
Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
Blood Transfusion
Blood transfusion is a critical medical procedure that saves lives and treats various medical conditions. It involves transferring blood from a donor to a recipient. This process requires a thorough understanding of the ABO blood group system and its associated antigens and antibodies.
Blood Transfusion Overview
A blood transfusion is a medical procedure used to replace blood lost due to injury, surgery, or to treat conditions such as anemia or cancer. During a transfusion, donor blood is...
Blood Transfusion Overview
A blood transfusion is a medical procedure used to replace blood lost due to injury, surgery, or to treat conditions such as anemia or cancer. During a transfusion, donor blood is...
Venous Thrombosis I: Introduction
Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
Hypoglycemia
Hypoglycemia is a blood glucose level below 70 mg/dL. It commonly occurs in individuals using insulin or insulin-secreting drugs, but may also arise in non-diabetic conditions. People with type 1 diabetes are at the highest risk because they depend on exogenous insulin. People with type 2 diabetes are also at risk, especially when treated with insulin or medications such as sulfonylureas, which increase insulin release regardless of blood glucose levels. It develops when insulin levels exceed...
Diabetic Retinopathy
DefinitionDiabetic retinopathy is a microvascular complication of diabetes affecting the retinal blood vessels.Risk FactorsDiabetic retinopathy is present in almost all individuals with type 1 diabetes and more than 60% of those with type 2 diabetes after two decades of disease.The risk increases with poor glycemic control, hypertension, dyslipidemia, smoking, pregnancy, and puberty.Although cataracts and glaucoma are also more frequent in people with diabetes, retinopathy remains the leading...


