不稳定血红蛋白(UHb)病是因遗传性缺陷致血红蛋白分子中某些氨基酸被替换或丢失,使分子的稳定性降低,易在体内变性沉淀形成Heinz小体(又称变性珠蛋白小体),并可引起溶血性贫血的一类血红蛋白分子病。首例是1952年由Cathie报告,被称为先天性Heinz小体溶血性贫血;1960年Frick才 Unstable hemoglobin (UHb) disease is due to genetic defects caused by some of the amino acids in hemoglobin molecules are replaced or lost, reducing the stability of the molecule, easy to precipitate in the body to form Heinz bodies (also known as denatured globin body) And can cause hemolytic anemia of a class of hemoglobin disease. The first case was reported by Cathie in 1952 and was called congenital Heinz’s body hemolytic anemia; in 1960 Frick