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血清Ⅲ型前胶原肽水平与骨髓化生的骨髓纤维化疾病活动度相关。

Serum procollagen-III-peptide level correlates with disease activity in myelofibrosis with myeloid metaplasia.

作者信息

Barosi G, Costa A, Liberato L N, Polino G, Spriano P, Magrini U

机构信息

Dipartimento di Medicina Interna e Terapia Medical (Sez. Clinica Medica II), IRCCS Policlinico S. Matteo, Pavia, Italy.

出版信息

Br J Haematol. 1989 May;72(1):16-20. doi: 10.1111/j.1365-2141.1989.tb07644.x.

DOI:10.1111/j.1365-2141.1989.tb07644.x
PMID:2736239
Abstract

In 78 patients with myelofibrosis with myeloid metaplasia (MMM) the serum procollagen III peptide activity (s-PIIIP) had a higher mean value than in 22 normal adult volunteers (22.5 v. 10 ng/ml). Nevertheless, 21.8% of the patients had s-PIIIP within the 95th percentile of the normal control group (16 ng/ml): with respect to those whose concentrations exceeded this limit, patients with normal s-PIIIP levels were younger (55.8 v. 61.2 years), had a higher Hb value (12.3 v. 10.5 g/l), a lower serum ferritin level (106 v. 464 micrograms/l) and a higher platelet count (390 v. 216 x 10(9)/l). In the overall patient population, s-PIIIP was significantly higher in those with symptoms of active disease (fever, sweating, weight loss) than in subjects with non-active disease (28 v. 16.9 ng/ml). At univariate analysis s-PIIIP correlated (at the 5% level) with increasing WBC, serum ferritin and number of transfusions and with decreasing Hb and platelet count. At multivariate analysis increasing WBC, serum ferritin and age proved to be independently associated with s-PIIIP. No relationship was found between the s-PIIIP level and morphometric grading of bone marrow fibrosis, megakaryocyte number, or lymphoid infiltration. Longitudinal studies showed that s-PIIIP increased with disease progression. The conclusion of the study is that s-PIIIP correlates more with overall disease activity than with the extent of bone marrow fibrosis.

摘要

在78例骨髓纤维化伴髓外化生(MMM)患者中,血清前胶原III肽活性(s - PIIIP)的平均值高于22名正常成年志愿者(分别为22.5和10 ng/ml)。然而,21.8%的患者s - PIIIP处于正常对照组第95百分位数(16 ng/ml)范围内:与浓度超过此限值的患者相比,s - PIIIP水平正常的患者更年轻(55.8岁对61.2岁),血红蛋白值更高(12.3对10.5 g/l),血清铁蛋白水平更低(106对464 μg/l),血小板计数更高(390对216×10⁹/l)。在整个患者群体中,有活动性疾病症状(发热、出汗、体重减轻)的患者s - PIIIP显著高于无活动性疾病的患者(28对16.9 ng/ml)。单因素分析显示,s - PIIIP与白细胞计数增加、血清铁蛋白和输血次数增加以及血红蛋白和血小板计数降低(在5%水平)相关。多因素分析表明,白细胞计数增加、血清铁蛋白和年龄被证明与s - PIIIP独立相关。未发现s - PIIIP水平与骨髓纤维化的形态学分级、巨核细胞数量或淋巴细胞浸润之间存在关联。纵向研究表明,s - PIIIP随疾病进展而增加。该研究的结论是,s - PIIIP与整体疾病活动的相关性大于与骨髓纤维化程度的相关性。

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Bone geometry, bone mineral density, and micro-architecture in patients with myelofibrosis: a cross-sectional study using DXA, HR-pQCT, and bone turnover markers.骨髓纤维化患者的骨几何形态、骨密度和微观结构:一项使用双能X线吸收法(DXA)、高分辨率外周定量CT(HR-pQCT)和骨转换标志物的横断面研究。
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Pathogenesis of idiopathic myelofibrosis: role of growth factors.
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J Clin Pathol. 1992 Jun;45(6):461-4. doi: 10.1136/jcp.45.6.461.