Abstract
Patients with the non-deletion genotype of HbH disease have higher proportions of HbH and more severe tissue hypoxia, than patients with the deletion genotype. As their red cells contain mainly two Hb species, HbH and HbA, one could exploit the high proportions of HbA by lowering its oxygen affinity and therefore increase the oxygen delivery of the red cells. Allosteric effectors which produce a low affinity Hb may be useful in this case. We investigated the effect of a bezafibrate derivative, RSR-4, on the oxygen affinity of red cells and purified hemolysates containing HbA and HbH. This allosteric effector crosses red cell membrane and binds reversibly to theα-chains of deoxy-Hb decreasing its oxygen affinity. The blood used was obtained from a patient with HbH disease (αTSaudi homozygote) whose HbH level was 33.5%, as measured by ce-HPLC. Oxygen binding studies were performed in red cells and purified hemolysates using the Hemox-Analyzer. The results showed that:
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