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原发性血小板增多症患者的血小板c-mpl表达失调,但这并无诊断价值。

Platelet c-mpl expression is dysregulated in patients with essential thrombocythaemia but this is not of diagnostic value.

作者信息

Harrison C N, Gale R E, Pezella F, Mire-Sluis A, MacHin S J, Linch D C

机构信息

Department of Haematology, University College London.

出版信息

Br J Haematol. 1999 Oct;107(1):139-47. doi: 10.1046/j.1365-2141.1999.01667.x.

Abstract

Essential thrombocythaemia (ET) can be difficult to discriminate from an occult case of reactive thrombocytosis (RT). Since thrombopoietin (TPO) is the primary regulator of thrombopoiesis, we have investigated whether levels of TPO and/or its receptor, c-mpl, are of value in the differential diagnosis of ET. Plasma TPO levels in patients with ET, RT and other myeloproliferative disorders (MPDs) did not differ significantly from normal controls. However, surface c-mpl expression was significantly reduced in platelets from 18 ET patients, 0-65.5% of controls (P < 0.001). Immunoblots on five of these and five additional patients were consistent with absent or reduced c-mpl protein levels. The surface c-mpl expression results were significantly different from those in eight RT patients (21. 3-95.5%, P = 0.0015), but there was considerable overlap between the two groups and a reduced level was not restricted to ET. Furthermore, c-mpl expression in ET patients was not different from eight patients with other MPDs (0-87.6%, P = 0.06), nor could it differentiate between ET patients with monoclonal and polyclonal haemopoiesis. Although a low or absent c-mpl level is suggestive of a primary rather than a secondary thrombocytosis, it is insufficiently discriminatory to be used as a diagnostic marker for ET.

摘要

原发性血小板增多症(ET)可能难以与隐匿性反应性血小板增多症(RT)病例相鉴别。由于血小板生成素(TPO)是血小板生成的主要调节因子,我们研究了TPO及其受体c-mpl的水平在ET的鉴别诊断中是否具有价值。ET、RT及其他骨髓增殖性疾病(MPD)患者的血浆TPO水平与正常对照组相比无显著差异。然而,18例ET患者血小板表面c-mpl表达显著降低,为对照组的0 - 65.5%(P < 0.001)。其中5例患者及另外5例患者的免疫印迹结果与c-mpl蛋白水平缺失或降低一致。表面c-mpl表达结果与8例RT患者(21.3 - 95.5%,P = 0.0015)的结果有显著差异,但两组之间有相当程度的重叠,且水平降低并不局限于ET。此外,ET患者的c-mpl表达与8例其他MPD患者无差异(0 - 87.6%,P = 0.06),也无法区分单克隆和多克隆造血的ET患者。尽管c-mpl水平低或缺失提示为原发性而非继发性血小板增多症,但它作为ET的诊断标志物的鉴别能力不足。

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