“…Hemoglobin Bushwick confers a significant but compensated hemolytic disorder that is considerably milder than some other kinds of US‐Hb. For example, Hb Hirosaki, 10 which was found for the first time by us, results in moderate anemia and jaundice. The present case, in contrast, usually showed no anemia and had only slight jaundice resulting from mild compensated hemolysis.…”
“…Hemoglobin Bushwick confers a significant but compensated hemolytic disorder that is considerably milder than some other kinds of US‐Hb. For example, Hb Hirosaki, 10 which was found for the first time by us, results in moderate anemia and jaundice. The present case, in contrast, usually showed no anemia and had only slight jaundice resulting from mild compensated hemolysis.…”
A 48-year-old man had a 30-year history of hemolytic anemia of undetermined cause. Spherocytes were not observed, osmotic fragility was normal, and red cell enzyme activities were normal. His brother and daughter also had hemolytic anemia. The brother had previously undergone splenectomy, and the anemia had been ameliorated. In the proband and daughter, no abnormal hemoglobin was apparent in the results of isoelectric focusing and DEAE anion-exchange high-performance liquid chromatography analyses. On evaluation with the isopropanol test, unstable hemoglobin was not observed in the proband but was detected in the daughter. There was also a decreased ratio of 3 globin/3 globin chain production. Analysis of the 32 gene demonstrated the presence of a mutation (alpha43 [CE1] Phe --> Leu), hemoglobin Hirosaki.
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