Tangsinmankong N, Bahna S L, Good R A
Department of Allergy and Immunology, University of South Florida/All Children's Hospital, St. Petersburg 33701, USA.
Ann Allergy Asthma Immunol. 2001 Nov;87(5):362-9; quiz 370, 423. doi: 10.1016/S1081-1206(10)62915-8.
This review is intended to provide an outline for the evaluation of patients suspected of having immunodeficiency, a problem that is frequently encountered in clinical practice.
Information was obtained through a MEDLINE literature search as well as from standard textbooks in immunology. Also included is information that reflects the authors' clinical experience in the field.
In general clinical practice, many physicians feel inadequate to evaluate patients with suspected immune deficiencies. They also think that the process of evaluation is time-consuming, which results in misdiagnosis of a substantial percentage of such disorders. Hence, the prevalence of immunodeficiency disorders is much higher than generally thought. At present, there are >80 unique primary immunodeficiency conditions and >50 syndromes that are associated with various immunologic defects. The prevalence of secondary immunodeficiency has also been increasing because of the tragic epidemic of HIV infection, more usage of immunosuppressive medications and bone marrow stem cell transplantation, and the severe degree of malnutrition in underdeveloped countries. It is necessary for clinicians, particularly the specialists in allergy and immunology, to be able to evaluate the status of the immune system.
Very valuable information can be gathered from the medical history and physical examination that may exclude or increase the suspicion of immunologic defect. Laboratory tests can then be appropriately selected to define the specific defect. Once the diagnosis has been settled, proper medical management can be instituted with subsequent improvement in morbidity and mortality of such disorders.
本综述旨在为疑似免疫缺陷患者的评估提供一个概述,免疫缺陷问题在临床实践中经常遇到。
通过MEDLINE文献检索以及免疫学科的标准教科书获取信息。还包括反映作者在该领域临床经验的信息。
在一般临床实践中,许多医生认为自己没有足够能力评估疑似免疫缺陷的患者。他们还认为评估过程耗时,这导致相当比例的此类疾病被误诊。因此,免疫缺陷疾病的患病率远高于普遍认知。目前,有超过80种独特的原发性免疫缺陷疾病以及超过50种与各种免疫缺陷相关的综合征。由于艾滋病毒感染的悲剧性流行、免疫抑制药物和骨髓干细胞移植的更多使用以及欠发达国家严重的营养不良状况,继发性免疫缺陷的患病率也在上升。临床医生,尤其是过敏和免疫学专家,有必要能够评估免疫系统的状态。
从病史和体格检查中可以收集到非常有价值的信息,这些信息可能排除或增加对免疫缺陷的怀疑。然后可以适当选择实验室检查来确定具体缺陷。一旦确诊,就可以进行适当的医疗管理,从而改善此类疾病的发病率和死亡率。