Clinical Features and Genotypes of Patients with Hemoglobin H Disease in Taiwan.

BACKGROUND: The genetic background of patients with hemoglobin (Hb) H disease in Taiwan has been investigated; however, the clinical features and treatment outcomes were not reported. OBJECTIVE: To analyze the clinical features and genotypes of patients with HbH who reside in Taiwan. METHODS: We conducted a retrospective analysis of the clinical and molecular characteristics of 38 patients with HbH disease who were undergoing treatment at Kaohsiung Medical University Hospital, Taiwan. RESULTS: Initial Hb levels were lower and the numbers of patients requiring iron-chelation therapy were higher in the nondeletional HbH group than in the deletional HbH group (P <.05). Compared with the healthy population, the patients with HbH disease exhibited short body length, low body weight, and low body mass index (BMI). CONCLUSIONS: Patients with nondeletional HbH disease had lower Hb levels and a higher requirement for splenectomy and iron-chelation therapy than did those with deletional HbH disease. Also, growth status was compromised in patients with HbH disease.

Lipid peroxidation and antioxidative status in beta-thalassemia major patients with or without hepatitis C virus infection.

BACKGROUND: Information pertaining to the lipid peroxidation and antioxidative status of patients with beta-thalassemic major, with or without hepatitis C virus infection, has been scanty. METHODS: We report here the results of our efforts in the evaluation of lipid peroxidative status, antioxidants, and vitamin A, E and C levels in the sera of a group of patients (n=42) with transfusion-dependent beta-thalassemic major with or without HCV infection. RESULTS: Firstly, plasma thiobarbituric acid reactive substance, a lipid peroxidation product, in these patients was found to be increased significantly when compared to the disease-free controls (p<0.05). Conversely, levels of plasma vitamins A, E and C were all shown to be drastically reduced as compared to the disease-free controls (p<0.01). In parallel with these data, we also found that HCV infection did play some role in aggravating the depletion of plasma vitamin E and C levels in the beta-thalassemic patients. In contrast, HCV infection did not seem to alter the levels of reduced glutathione (GSH) as well as antioxidant enzyme activities including superoxide dismutase and GSH peroxidase. CONCLUSIONS: Taken together, our data indicate that excessive lipid peroxidation and a profound depletion of plasma vitamin A, E and C levels exist in patients with beta-thalassemic major. These data suggest that antioxidant supplementation to the patients for the purpose of alleviating the oxidative stress may be warranted.