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真性红细胞增多症向骨髓纤维化的发展。通过前胶原III氨基端前肽测定进行监测

[Development of polycythemia to myelofibrosis. Monitoring by the assay of the procollagen III amino-propeptide].

作者信息

Arrago J P, Poirier O, Najean Y

出版信息

Presse Med. 1984 Nov 10;13(40):2429-32.

PMID:6239221
Abstract

The conventional methods used in the follow-up of myelofibrosis being both invasive and expensive, we propose an easy and sensitive immunoassay of the procollagen III aminoterminal peptide (propeptide) which is separated and released in serum during synthesis of collagen III by bone marrow fibroblasts. This propeptide was assayed in the sera of 25 healthy adults (controls), 48 patients with polycythaemia vera and 20 patients with myelofibrosis (secondary to polycythaemia in 14). Significant differences in mean values were found between controls (7.6 +/- 2.1 ng/ml), patients with polycythaemia (9.9 +/- 4.3 ng/ml) and patients with myelofibrosis (38.1 ng/ml). There was some overlapping between values in controls and in patients with polycythaemia, partly due to the fact that 12 of these 48 patients without reticulin bone marrow fibrosis had normal serum propeptide levels. Propeptide values in patients with myelofibrosis were much scattered (range: 13-103 ng/ml). Moreover, values above 25 ng/ml were associated with myelofibrosis of recent onset (less than or equal to 2 years) and values below 25 ng/ml with myelofibrosis of more than 4 years' duration. The procollagen III aminoterminal peptide immunoassay therefore is a non-invasive and sensitive method for accurate assessment of bone marrow progressive involvement in myeloproliferative diseases associated with myelofibrosis. It could also be used to evaluate the effectiveness of antifibrosing agents.

摘要

用于骨髓纤维化随访的传统方法既具有侵入性又昂贵,我们提出了一种简便且灵敏的免疫测定法,用于检测前胶原III氨基末端肽(前肽)。在骨髓成纤维细胞合成III型胶原期间,该前肽在血清中分离并释放。我们对25名健康成年人(对照组)、48名真性红细胞增多症患者和20名骨髓纤维化患者(14例继发于真性红细胞增多症)的血清进行了该前肽检测。结果发现,对照组(7.6±2.1 ng/ml)、真性红细胞增多症患者(9.9±4.3 ng/ml)和骨髓纤维化患者(38.1 ng/ml)的平均值存在显著差异。对照组和真性红细胞增多症患者的值有部分重叠,部分原因是这48例无网状纤维骨髓纤维化的患者中有12例血清前肽水平正常。骨髓纤维化患者的前肽值分布较为分散(范围:13 - 103 ng/ml)。此外,前肽值高于25 ng/ml与近期发病(小于或等于2年)的骨髓纤维化相关,而低于25 ng/ml则与病程超过4年的骨髓纤维化相关。因此,前胶原III氨基末端肽免疫测定法是一种用于准确评估骨髓在与骨髓纤维化相关的骨髓增殖性疾病中进展性受累情况的非侵入性且灵敏的方法。它还可用于评估抗纤维化药物的疗效。

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