(kŏn-jen'i-tăl hīnz bod'ē hē'mō-lit'ik ă-nē'mē-ă) Any of several inherited disorders of hemoglobin synthesis in which an amino acid substitution results in faulty molecular configuration, with defective binding of heme to globin. Denatured hemoglobin forms Heinz bodies, which damage the erythrocyte membrane and promote premature hemolysis.