Effect of pyrexia in the formation of intraerythrocytic inclusion bodies and vacuoles in haemolytic crisis of haemoglobin H disease

Abstract

Clinical aspects and in vivo formation of inclusion bodies and vacuoles in red cells of patients with haemoglobin H disease were studied and compared during steady state and after pyrexia of acute infections. During period of pyrexia, there were haemolytic crises with significant drops of the steady-state haemoglobin levels of 8.6 +/- 0.9 g/dl to 5.6 +/- 1.7 g/dl. The phenomenon was observed with splenic enlargement and significant increase of inclusion body formation. The findings suggested that inclusion body formation in red cells during pyrexia of acute infections played an important role in haemolytic crisis of haemoglobin H disease, probably by causing these cells to be sequestered and destroyed in the spleen.