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贫血时的血红素分解代谢、一氧化碳生成与红细胞存活

Heme catabolism, carbon monoxide production and red cell survival in anemia.

作者信息

Lundh B, Cavallin-Ståhl E

出版信息

Acta Med Scand. 1975 Mar;197(3):161-71. doi: 10.1111/j.0954-6820.1975.tb04898.x.

Abstract

Total heme catabolism has been studied through measurement of the endogenous production of carbon monoxide (VCO) in 8 patients with hemolysis, 7 with hypoproliferative anemia, 10 with refractory anemia and hypercellular bone marrow and 7 with splenomegaly, 6 of whom had myeloid metaplasia. Simultaneously, catabolism of circulating red cell hemoglobin heme (Vheme-c) was measured through labelling of the red cells with 51Cr, and the VCA/Vheme-c ratio was calculated for each patient. From a control group it was calculated that this ratio should vary around 1.5. Since no isotope studies were performed in the control group, no range could be defined. Among patients with hemolysis the VCO/Vheme-c ratio was found to vary between 1.3 and 1.8 except in 2 cases of paroxysmal nocturnal hemoglobinuria (PNH) and PNH?, respectively, in whom the ratios were found to be 0.6 and 0.7 suggesting some heme catabolism without corresponding CO formation. In the hypoproliferative group the ratio varied between 1.2 and 1.8 except in one patient treated with androgens, in whom the ratio was found to be 2.9, suggesting increased extraerythrocytic heme turnover. In patients with myeloid metaplasia the ratio varied between 1.3 and 1.8. On the other hand, the ratio varied getween 2.4 and 3.0 among patients with refractory anemia and hypercellular bone marrow, thus confirming earlier findings that in this type of anemia turnover of bone marrow heme is markedly increased. A significant correlation was found between VCO and initial morning COHb%(r equals 0.84). The conclusions drawn are (a) that Vheme-c sometimes represents less than 50% of total heme turnover and (b) that COHb and/or VCO reflect total heme turnover except in patients with blood loss or intravascular hemolysis with hemoglobinuria.

摘要

通过测量8例溶血患者、7例增生低下性贫血患者、10例难治性贫血伴骨髓细胞增多患者以及7例脾肿大患者(其中6例有骨髓化生)内源性一氧化碳生成量(VCO),对总血红素分解代谢进行了研究。同时,通过用51Cr标记红细胞来测量循环红细胞血红蛋白血红素的分解代谢(Vheme-c),并计算每位患者的VCA/Vheme-c比值。从对照组计算得出该比值应在1.5左右波动。由于对照组未进行同位素研究,所以无法确定其范围。在溶血患者中,VCO/Vheme-c比值在1.3至1.8之间变化,但分别有2例阵发性夜间血红蛋白尿(PNH)和PNH?患者,其比值分别为0.6和0.7,提示存在一些血红素分解代谢但无相应的一氧化碳生成。在增生低下组中,该比值在1.2至1.8之间变化,但有1例接受雄激素治疗的患者,其比值为2.9,提示红细胞外血红素周转增加。在骨髓化生患者中,该比值在1.3至1.8之间变化。另一方面,难治性贫血伴骨髓细胞增多患者的比值在2.4至3.0之间变化,从而证实了早期的发现,即这种类型的贫血中骨髓血红素周转明显增加。发现VCO与清晨初始COHb%之间存在显著相关性(r等于0.84)。得出的结论是:(a)Vheme-c有时占总血红素周转的比例不到50%;(b)除失血或伴有血红蛋白尿的血管内溶血患者外,COHb和/或VCO反映总血红素周转。

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