抄録
More than 38 human hemoglobin variants and 63 families with thalassemia have been reported in Japan.
The present report have summarized following main aspects:
1. The serial consequences on seven unstable variants in relation to molecular abnormality, denaturation pathway of hemoglobin, erythrocyte destruction and clinical and hematological features have been discussed.
2. Also, the efficiency of splenectomy was various among patients with unstable variants. It was suggested that ferrokinetic and red cell survival studies which locate the organs destructing the red cells are important indices for the prediction of the effect of splenectomy.
3. Diagnostic significance on the estimation of globin synthesis in thalassemic patients was confirmed.
