Schultz J C
Clinical Laboratories, University of Wisconsin Medical School, Madison 53792, USA.
J Clin Lab Anal. 1995;9(6):366-74. doi: 10.1002/jcla.1860090606.
Sickle cell disease covers a group of conditions in which pathology may be attributed to the presence of sickle hemoglobin (HbS). The identification of HbS and other variants including those in combination with HbS is commonly achieved by cellulose acetate electrophoresis at alkaline pH. Because many hemoglobin variants with similar charges have similar electrophoretic migration patterns, they are difficult to differentiate by electrophoresis. The HemoCard assays address this concern through the use of monoclonal antibodies capable of specifically recognizing the unique amino acid substitution in the variant hemoglobin. The panel of HemoCard monoclonal antibodies confirms the absence and presence of HbA, HbC, HbE, HbS, and other sickling hemoglobin variants. The combination of alkaline cellulose acetate electrophoresis and HemoCard assays allows the technologist to reach a final conformation of both common and much less common sickle cell disease genotypes, combinations of HbS with other hemoglobins that ordinarily do not produce sickle cell disease, and other clinically important hemoglobinopathies including HbE/beta-thalassemia and hemoglobin C disease.
镰状细胞病涵盖了一组病症,其病理可能归因于镰状血红蛋白(HbS)的存在。HbS及其他变体(包括与HbS组合的变体)的鉴定通常通过在碱性pH条件下的醋酸纤维素电泳来实现。由于许多电荷相似的血红蛋白变体具有相似的电泳迁移模式,因此难以通过电泳进行区分。HemoCard检测通过使用能够特异性识别变体血红蛋白中独特氨基酸取代的单克隆抗体来解决这一问题。HemoCard单克隆抗体组可确认HbA、HbC、HbE、HbS及其他镰状血红蛋白变体的有无。碱性醋酸纤维素电泳和HemoCard检测相结合,使技术人员能够最终确定常见和罕见的镰状细胞病基因型、HbS与通常不会导致镰状细胞病的其他血红蛋白的组合,以及其他具有临床重要性的血红蛋白病,包括HbE/β地中海贫血和血红蛋白C病。