Najean Y, Cacchione R, Castro-Malaspina H, Dresch C
Br J Haematol. 1978 Oct;40(2):205-17. doi: 10.1111/j.1365-2141.1978.tb03658.x.
Eighty-three patients with myelofibrosis have been studied by erythrokinetics and have been followed up until death or for at least 12 months. Because of a large plasma volume the venous haematocrit gives only a poor idea of the red blood cell volume. The red cell survival was reduced in the majority of cases but significant haemolysis was rare. The amount of haemolysis of autologous and isologous red cells was similar, suggesting an extra-corpuscular origin for the haemolysis. Plasma iron turnover was always increased, sometimes markedly, but red cell iron incorporation was reduced in 70% of cases, indicating ineffective erythropoiesis. Surface counting showed an absence on diminution of sacral iron fixation and a rapid and marked splenic uptake in more than 90% of the cases; uptake of iron by the liver occurred in half the cases, usually not very high; iron release from the spleen was absent or reduced in 67% of the cases. The degree of ineffective erythropoiesis as measured by radio-iron incorporation and release by the spleen, the amount of haemolysis, and the red cell volume were strongly correlated with prognosis. These factors enabled a more precise prediction to be made of the clinical outcome in the 2 years following the study, than the clinical data alone. A prospective study might show whether erythrokinetic studies are also useful in determining the choice of treatment.
对83例骨髓纤维化患者进行了红细胞动力学研究，并对其进行随访直至死亡或至少随访12个月。由于血浆量较大，静脉血细胞比容只能粗略反映红细胞体积。大多数病例中红细胞存活期缩短，但明显溶血罕见。自体和同种异体红细胞的溶血量相似，提示溶血源于细胞外因素。血浆铁周转率总是升高，有时显著升高，但70%的病例红细胞铁摄取减少，表明存在无效造血。体表计数显示，超过90%的病例骶部铁固定无减少，脾脏摄取迅速且显著；半数病例肝脏有铁摄取，通常摄取量不高；67%的病例脾脏无铁释放或铁释放减少。通过放射性铁摄取和脾脏释放量测定的无效造血程度、溶血量和红细胞体积与预后密切相关。与仅依靠临床数据相比，这些因素能更精确地预测研究后2年的临床结局。一项前瞻性研究可能会表明红细胞动力学研究在确定治疗选择方面是否也有用。
