Molecular characterization and functional studies on Hb Kempsey, beta 99 (G-1) Asp----Asn, a high-oxygen affinity variant.

Background: Hb Kempsey (beta 99 Asp----Asn) is a high-oxygen affinity hemoglobin, never before reported in Italy, associated with secondary erythrocytosis. It has been found in the heterozygous state in two subjects from the same family originating from the Verona area in Northern Italy.

Methods: The abnormal hemoglobin was studied both at the protein and at the DNA level.

Further studies on oxygen loading in blood from anemic patients.

Functional parameters of the whole blood oxygen loading from 66 anemic subjects, compared with those from 66 normal adult of both sexes display the following behaviour: a) Adult males and females differ for p50 values, which are higher in females because of their lower Hb level. This difference is already well known and appears to be statistically significant. b) The same difference is amplified in anemic subjects, where also pH lowering contributes to the right-shift of p50.

A follow-up on functional parameters of blood stored in ACD at +4 degrees C.

The main functional parameters of blood stored at +4 degrees C in ACD, according to the common transfusional practice, have been carefully followed in the course of 40 days. The expected depletion of DPG takes place within 10 days, but apparently, no increase of the Hb affinity towards oxygen is observed in this period (or later), because pH lowering acts in the opposite direction during the same time. However, the intrinsic increased affinity of Hb is promptly revealed if the "actual" pHs are corrected at the standard value of 7.