Recent interest in therapies for sickle cell anemia based on elevating fetal Hb has made accurate estimates of the sparing effect of fetal Hb (Hb F) and other non-sickle Hbs on sickle Hb (Hb S) polymerization essential. We have developed a technique, using HbCO as surrogate for HbO2, that enables us to assess the solubility of Hb S as a function of ligand saturation under conditions that mimic ...

Hb Hoshida (␤43Glu3 Gln). All 4 patients with abnormal peaks at the S window on HPLC (mean retention time, 0.91 min) had Hb Queens (␣1 34Leu3 Arg). All 10 patients with variant peaks were heterozygous, and they had abnormal bands within the Hb S/Hb G/Hb D area on cellulose acetate Hb electrophoresis. Hb G Coushatta has been found in Koreans, Chinese, and in some Japa-nese families (6). This var...

BACKGROUND In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/β(0)-thalassemia. OBJECTIVE To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia. METHODS...

CASE DESCRIPTION A sample submitted for hemoglobin (Hb) evaluation on a 5-day-old premature male infant with intestinal perforation, intraventricular hemorrhage, anemia, and sepsis contained 61.1% Hb A, 23.7% Hb F, 2.1% Hb A2, and peaks of 8.2% and 5.2% in the P2 and P3 regions of the Bio-Rad Variant II HPLC thalassemia assay (Fig. 1). Isoelectric focusing showed Hb A, Hb F, Hb A2, and a band c...

A recombinant hemoglobin containing Val beta 7 (Hb beta E7V) was engineered and expressed in yeast to evaluate amino acid specificity of the Glu beta 6-->Val mutation (Hb beta E6V) in promoting polymer formation of deoxyhemoglobin. The purified CO Hb beta E7V migrated as a single band on electrophoresis with a slightly decreased positive charge compared with CO Hb S. The oxygen affinity of Hb b...

Since the observation of a “fast-moving” hemoglobin (Hb) in diabetic blood specimens by Rahbar in 1968 [1] and the subsequent structural identification of the glucose-“modified” Hb [2], the measurement of erythrocyte glycoHb (Hb A1c) has served as the monitor for long-term glucose control for patients with diabetes mellitus [3]. Column chromatography was one of the first methodologies used for ...

Factors contributing to the clinical differences between sickle cell-hemoglobin C disease (SC) and the benign sickle cell trait (AS) include the higher proportion of hemoglobin (Hb) S and the higher cell Hb concentrations in SC compared with AS red cells. Reports differ, however, about whether Hb C copolymerizes more than Hb A with Hb S when measured by minimum gelling concentrations (MGCs) and...