Hemichromes in Single Inclusion Bodies in Red Cells of Beta Thalassemia

Inclusion bodies in the red cells of patients with thalassemia syndromes may result from precipitation of those hemoglobin subunits that are produced in relative excess. In hemoglobin H disease, a form of α-thalassemia, the inclusions are precipitated β-subunits, while in the β-thalassemias they are α-subunits. It has been shown that the inclusions in hemoglobin H disease have the spectral characteristics of hemichrome. In the present study, microspectrophotometric examination of single inclusion bodies in ghosts prepared from the red cells of two patients with β-thalassemia major revealed the absorption spectrum of hemichrome. These results suggest that hemichrome formation is an important pathogenetic event in the production of red cell inclusions and consequent hemolysis in the thalassemias.