Osifo B O, Adeyokunnu A, Parmentier Y, Gerard P, Nicolas J P
Scand J Haematol. 1983 Feb;30(2):135-40. doi: 10.1111/j.1600-0609.1983.tb01459.x.
The unsaturated vitamin B12 binding capacity (UBBC) of serum, the three transcobalamins (TC I, TC II, TC III), and the total leucocyte and neutrophil counts have been studied in paediatric patients with sickle cell disease (SCD). Increase in the level of the binding capacities of TC I and TC II with concomitant increase of UBBC was observed in these children who also had increased total white blood and neutrophil counts. There was a significant reduction in the level of endogenous B12. These abnormalities are discussed in relation to the deficiency of the splenic reticuloendothelial function, immunologic defect, hepatic degenerative changes and aplastic crisis observed in SCD. These results lend support to the view that transcobalamins are involved in the defence mechanism of the body. The significant reduction in serum cobalamin in SCD suggests a higher demand on this vitamin for metabolic functions, especially for the sparing of folate.
对镰状细胞病（SCD）患儿的血清不饱和维生素B12结合能力（UBBC）、三种转钴胺素（TC I、TC II、TC III）以及白细胞和中性粒细胞总数进行了研究。在这些患儿中，观察到TC I和TC II结合能力水平升高，同时UBBC增加，且白细胞和中性粒细胞总数也增加。内源性B12水平显著降低。结合SCD中观察到的脾网状内皮功能缺陷、免疫缺陷、肝脏退行性改变和再生障碍危象，对这些异常情况进行了讨论。这些结果支持了转钴胺素参与机体防御机制的观点。SCD中血清钴胺素的显著降低表明对这种维生素的代谢功能，尤其是对叶酸的节约有更高的需求。
