Different patterns of intraerythrocytic inclusion body distribution in the two types of haemoglobin H disease. An ultrastructural study.

Electron microscopic examination of intraerythrocytic inclusion bodies induced by methylene blue was carried out in 7 non-splenectomized patients with haemoglobin (Hb) H disease. In classical Hb H disease with the genotype of alpha-thalassaemia 1/alpha-thalassaemia 2 the inclusion bodies were mostly membrane-associated. In contrast, in Hb H disease of alpha-thalassaemia 1/Hb Constant Spring genotype the majority of inclusion bodies were in a floating stage. Possible mechanisms for the unexpected difference are discussed.