Hemoglobin H disease in Guangxi province, Southern China: clinical review of 357 patients.

摘要

The clinical characteristics of 357 patients with hemoglobin H (HbH) disease from the Guangxi province of Southern China were studied. One hundred and ninety-one (53.3%) patients were diagnosed with HbH-Constant Spring, 19 were diagnosed with HbH Westmead. Ten patients were shown to have coinherited HbH-Constant Spring/QS with a beta-thalassemia mutation. Coinheritance of the beta-thalassemia gene does not alleviate anemia (8.2 +/- 2.3 vs. 7.6 +/- 1.7 g/dl, p = 0.276), or influence age at diagnosis (20.2 +/- 19.6 vs. 12.9 +/- 11.0 years, p = 0.276). Ferritin levels were significantly higher in the group of patients with the nondeletional form of the disease (475 +/- 719 vs. 249 +/- 264 ng/ml, p = 0.005).