Hemoglobin Hasharon (α247 his(CD5)β2): a hemoglobin found in low concentration

Hemoglobin Hasharon in a Premature Infant with Hemolytic Anemia

“…Therefore the mutant hemoglobin was hemoglobin Hasharon, in which the aspartic acid at position 47 was replaced by histidine. This hemoglobin had been described previously in at least five other families, all of whom were of Jewish descent (3,7,26,28,29). There were n o known Jewish ancestors in this family.…”

“…Adult heterozygotes are usually asymptomatic and have normal hematologic values (4,7,26,29). However, Charache et al (4) described a 70-year-old heterozygote who had splenomegaly, gallstones, and a hemolytic anemia with a reticulocyte count of 5%.…”

“…Hasharon a chains with normal 0 chains must be only minimally decreased (4). However, the interaction of Hasharon a chains with y chains would be considerably less.…”

“…These combination of both could account for this lower than 50% subunits are much more susceptible t o denaturation than is concentration. Csarache et al (4) showed that heterozygotes native hemoglobin (10). The unstable mutant hemoglobins synthesize only 20-27% of their hemoglobin as Hasharon, have a greater tendency t o dissociate into subunits, and this which demonstrated that decreased synthesis determines the proportion of Hasharon in heterozygotes.…”

“…Knowing that the adult form of hemoglobin Hasharon is less stable than hemoglobin A, Charache et al (4) suggested that overt hemolytic anemia might result if the synthesis of hemoglobin Hasharon were increased. It may be that the hemolysis which occurred in our patient was due t o an increased rate of synthesis of the mutant a chain, and that hemolysis resolved as the rate of synthesis of the mutant decreased t o adult levels.…”