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儿童免疫性血小板减少性紫癜的诊断

Diagnosis of immune thrombocytopenic purpura in children.

作者信息

Geddis Amy E, Balduini Carlo L

机构信息

Department of Pediatrics, University of California, San Diego, California 92107, USA.

出版信息

Curr Opin Hematol. 2007 Sep;14(5):520-5. doi: 10.1097/MOH.0b013e3282ab98f2.

Abstract

PURPOSE OF REVIEW

This review updates the differential diagnosis between inherited and acquired immune thrombocytopenic purpura as well as clinical practice on the initial diagnosis of children with the disease.

RECENT FINDINGS

A diagnosis of immune thrombocytopenic purpura may be based on an evaluation of the history, physical findings such as petechiae, bruising and mucous membrane bleeding, examination of peripheral blood films stained with Wright's or May-Grünwald-Giemsa, determination of blood counts, platelet size and appearance. Recently, diagnostic assays have been developed to detect platelet-bound antibodies. The sensitivity of these assays, however, is suboptimal, with a positive predictive value of 80-83%. If the diagnosis of immune thrombocytopenic purpura is in question due to the presence of atypical features, or if a patient with findings typical of the disease does not respond to therapy, bone marrow aspiration and biopsy are indicated to confirm the diagnosis.

SUMMARY

The diagnosis of immune thrombocytopenic purpura is a process of elimination of other sources of thrombocytopenia. If the criteria discussed above are inconclusive and if the patient does not respond to therapy in 6-12 months (this is especially true in children) then a bone marrow aspiration is required to confirm the diagnosis, especially before initiating corticosteroid therapy.

摘要

综述目的

本综述更新了遗传性和获得性免疫性血小板减少性紫癜的鉴别诊断以及该疾病患儿初始诊断的临床实践。

最新发现

免疫性血小板减少性紫癜的诊断可基于病史评估、体格检查结果(如瘀点、瘀斑和黏膜出血)、用瑞氏或美-格二氏染色的外周血涂片检查、血细胞计数测定、血小板大小和外观。最近,已开发出检测血小板结合抗体的诊断检测方法。然而,这些检测方法的敏感性欠佳,阳性预测值为80%-83%。如果因存在非典型特征而对免疫性血小板减少性紫癜的诊断存疑,或者如果具有该疾病典型表现的患者对治疗无反应,则需进行骨髓穿刺和活检以确诊。

总结

免疫性血小板减少性紫癜的诊断是一个排除其他血小板减少病因的过程。如果上述标准无定论,且患者在6-12个月内对治疗无反应(儿童尤其如此),则需要进行骨髓穿刺以确诊,尤其是在开始使用皮质类固醇治疗之前。

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