Ruymann F B, Popejoy L A, Brouillard R B
Pediatr Res. 1978 Oct;12(10):1020-3. doi: 10.1203/00006450-197810000-00014.
A 13-year-old Thai female with hemoglobin E-beta-thalassemia was evaluated for anemia and splenomegaly. Globin chain synthesis in a whole cell system revealed an absence of betaA chains and excessive alpha chains. The alpha/betaE + gamma ratio was 1.26 in bone marrow and 1.90 in peripheral blood. The average gamma/betaE ratio in bone marrow and peripheral blood was 0.36 compared to peripheral blood concentrations of 49% hemoglobin E and 51% hemoglobin F. Homologous red cell 51Cr half-life increased from 22.7 days to 32.8 days after splenectomy. Total circulating hemoglobin increased from 112.9 to 149.7 g. Endogenous carbon monoxide productive (Vco) as a measure of total heme catabolism decreased from 2.00 to 1.54 mumol/hr/kg. Ineffective erythropoiesis was manifested by an increased Vco/Vheme-c ratio of 7.52.
一名患有血红蛋白E-β地中海贫血的13岁泰国女性因贫血和脾肿大接受评估。全细胞系统中的珠蛋白链合成显示缺乏βA链且α链过量。骨髓中α/βE + γ比值为1.26，外周血中为1.90。与外周血中49%的血红蛋白E和51%的血红蛋白F浓度相比，骨髓和外周血中的平均γ/βE比值为0.36。脾切除术后，同源红细胞51Cr半衰期从22.7天增加到32.8天。总循环血红蛋白从112.9克增加到149.7克。作为总血红素分解代谢指标的内源性一氧化碳生成量（Vco）从2.00降至1.54微摩尔/小时/千克。无效红细胞生成表现为Vco/Vheme-c比值增加至7.52。
