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骨髓纤维化伴髓外化生的红细胞生成:通过红细胞动力学识别不同类型的患者。

Erythropoiesis in myelofibrosis with myeloid metaplasia: recognition of different classes of patients by erythrokinetics.

作者信息

Barosi G, Cazzola M, Frassoni F, Orlandi E, Stefanelli M

出版信息

Br J Haematol. 1981 Jun;48(2):263-72. doi: 10.1111/j.1365-2141.1981.tb08459.x.

Abstract

A quantitative assessment of erythropoiesis was performed by means of a mathematical model of iron kinetics in 26 patients with myelofibrosis with myeloid metaplasia (MMM). A direct relationship between total erythropoiesis and red cell volume was revealed. This 'inverse' characteristic of erythropoietic control was the best marker of the proliferative nature of the disease. Three classes of patients were singled out by means of a cluster analysis of the erythrokinetic parameters. In class I (11 patients) the red cell volume was above the predicted normal in all but two patients. Erythropoiesis was sustained at 5--10 times the normal levels with a high degree of ineffective erythropoiesis. A fairly constant absence of erythroid activity over the sacral marrow was demonstrated. In class II most of the 12 patients had a decreased red cell volume. Erythropoiesis was sustained at 2--4 times the normal level with a high degree of peripheral haemolysis. Erythroid activity was recognized over the sacral area in all but two patients. The three patients of class III showed a pattern of erythroid failure and had the worst prognosis. It is suggested that these classes represent separate disease forms of MMM.

摘要

采用铁动力学数学模型对26例骨髓纤维化伴髓外化生(MMM)患者的红细胞生成进行了定量评估。结果显示总红细胞生成与红细胞体积之间存在直接关系。这种红细胞生成调控的“反向”特征是该疾病增殖性质的最佳标志。通过对红细胞动力学参数进行聚类分析,将患者分为三类。在I类(11例患者)中,除2例患者外,其余患者的红细胞体积均高于预测正常值。红细胞生成维持在正常水平的5 - 10倍,无效红细胞生成程度较高。骶骨骨髓区域几乎持续缺乏红系活性。在II类的12例患者中,大多数患者的红细胞体积降低。红细胞生成维持在正常水平的2 - 4倍,外周溶血程度较高。除2例患者外,其余患者骶骨区域均有红系活性。III类的3例患者表现为红系衰竭模式,预后最差。提示这些类别代表了MMM的不同疾病形式。

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