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原发性和继发性自身免疫性血小板减少症。一项血清学和临床分析。

Primary and secondary autoimmune thrombocytopenia. A serological and clinical analysis.

作者信息

Helmerhorst F M, van Leeuwen E F, Pegels J G, van der Plas-van Dalen C, Eengelfriet C P, von dem Borne A E

出版信息

Scand J Haematol. 1982 Apr;28(4):319-28. doi: 10.1111/j.1600-0609.1982.tb00533.x.

Abstract

In 357 thrombocytopenic patients, the autoimmune nature of the thrombocytopenia was established with the immunofluorescence test on paraformaldehyde-fixed platelets in suspension (PSIFT). In 142 patients, autoimmune thrombocytopenia (AITP) was accompanied by (an)other disease(s) and thus classified as secondary AITP. No significant difference was found in the distribution of the immunochemical characteristics of the autoantibodies between primary and secondary AITP. The results of survival studies with 51Cr-labelled platelets and organ sequestration measurements in 7 patients with idiopathic thrombocytopenia purpura (ITP) indicated that platelets with IgM autoantibodies were sequestered mainly in the spleen. An increased incidence of AITP was seen at 5 to 10 years of age, in the 3rd decade and in the 6th and 7th decades of life. AITP was slightly more common life in females. The following groups of accompanying diseases in 142 AITP patients were distinguished: autoimmune diseases of the blood, malignant diseases of the blood, generalized and organ-specific autoimmune diseases, carcinoma and a miscellaneous group of diseases. No significant differences were found in the immunochemical properties of the autoantibodies between the various categories of disease. In 7 cases, AITP was preceded by an established viral disease, in 1 case by lepra and in another by a vaccination. The PSIFT was found to be a suitable test for diagnosing AITP not only in idiopathic thrombocytopenia, but also in thrombocytopenia associated with another disease.

摘要

在357例血小板减少症患者中,通过对悬浮于多聚甲醛固定血小板上进行免疫荧光试验(PSIFT)确定了血小板减少症的自身免疫性质。在142例患者中,自身免疫性血小板减少症(AITP)伴有其他疾病,因此被归类为继发性AITP。原发性和继发性AITP之间自身抗体的免疫化学特征分布未发现显著差异。对7例特发性血小板减少性紫癜(ITP)患者进行的51Cr标记血小板存活研究和器官潴留测量结果表明,带有IgM自身抗体的血小板主要潴留在脾脏中。AITP在5至10岁、第三个十年以及第六和第七个十年发病率增加。AITP在女性中略为常见。对142例AITP患者的伴发疾病进行了如下分组:血液自身免疫性疾病、血液恶性疾病、全身性和器官特异性自身免疫性疾病、癌症以及其他杂类疾病。不同疾病类别之间自身抗体的免疫化学性质未发现显著差异。在7例患者中,AITP之前患有确诊的病毒性疾病,1例之前患有麻风病,另1例之前接受过疫苗接种。发现PSIFT不仅适用于诊断特发性血小板减少症中的AITP,也适用于诊断与其他疾病相关的血小板减少症。

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