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儿童慢性血小板减少症:无创方法在临床评估中的应用

Chronic thrombocytopenia of childhood: use of non-invasive methods in clinical evaluation.

作者信息

Rajantie J, Javela K, Joutsi-Korhonen L, Kekomäki R

机构信息

Helsinki University Central Hospital, Jorvi Hospital, Espoo, Finland.

出版信息

Eur J Haematol. 2004 Apr;72(4):268-72. doi: 10.1111/j.1600-0609.2004.00215.x.

Abstract

OBJECTIVES

An unselected group of 21 children with chronic thrombocytopenia was investigated to understand the patients' platelet abnormality better.

METHODS

Platelet counts, mean platelet volumes (MPV), membrane glycoproteins and Fcgamma receptor type IIA (FcgammaRIIA) polymorphism H131R, reticulated platelets (% RP), antiplatelet antibodies and plasma thrombopoietin (TPO) were measured.

RESULTS

Sixteen patients had idiopathic thrombocytopenic purpura (ITP) (group 1: platelets < 50 x 10(9)/L, n = 6; group 2: 50-99 x 10(9)/L, n = 4; group 3: 100-149 x 10(9)/L, n = 4; group 4: splenectomised patients with normal platelet counts, n = 2). Five patients had familial thrombocytopenia. Six healthy children were studied as a reference. In the 19 thrombocytopenic patients, the platelets were significantly larger and % RP and TPO levels were significantly higher than those in the controls. Increased megakaryocytosis at diagnosis was associated with larger MPV and higher % RP but not with platelet level or TPO. The % RP was remarkably high in all ITP patients of group 1 indicating a brisk production of platelets despite low peripheral count. In all patients with familial thrombocytopenia, TPO was increased suggesting that the syndrome was not because of defective TPO production. The distribution of FcgammaRIIA alleles in the patients was similar to that in the controls.

CONCLUSIONS

A combined application of % RP and TPO could be helpful in classifying patients with chronic thrombocytopenia into different categories. The observations may be of value in the clinical evaluation of ITP patients and lead to avoidance of invasive examinations at least in some patients.

摘要

目的

对一组未经挑选的21例慢性血小板减少症患儿进行研究,以更好地了解患者的血小板异常情况。

方法

检测血小板计数、平均血小板体积(MPV)、膜糖蛋白、Fcγ受体IIA(FcγRIIA)多态性H131R、网织血小板(% RP)、抗血小板抗体及血浆血小板生成素(TPO)。

结果

16例患者患有特发性血小板减少性紫癜(ITP)(第1组:血小板<50×10⁹/L,n = 6;第2组:50 - 99×10⁹/L,n = 4;第3组:100 - 149×10⁹/L,n = 4;第4组:脾切除术后血小板计数正常的患者,n = 2)。5例患者患有家族性血小板减少症。选取6名健康儿童作为对照。在19例血小板减少症患者中,血小板明显更大,% RP和TPO水平显著高于对照组。诊断时巨核细胞增多与较大的MPV和较高的% RP相关,但与血小板水平或TPO无关。第1组所有ITP患者中的% RP非常高,表明尽管外周血小板计数低,但血小板生成活跃。在所有家族性血小板减少症患者中,TPO升高,提示该综合征并非由于TPO生成缺陷所致。患者中FcγRIIA等位基因的分布与对照组相似。

结论

联合应用% RP和TPO有助于将慢性血小板减少症患者分为不同类别。这些观察结果可能对ITP患者的临床评估有价值,并至少在某些患者中避免进行侵入性检查。

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