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

Acta Haematol. 2010;124(2):86-91. doi: 10.1159/000314058. Epub 2010 Jul 15.

Abstract

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 β-thalassemia mutation. Coinheritance of the β-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).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Asian People / genetics
  • Asian People / statistics & numerical data
  • Child
  • Child, Preschool
  • China / epidemiology
  • Female
  • Ferritins / blood
  • Genetic Predisposition to Disease / ethnology
  • Genotype
  • Hemoglobin H / genetics*
  • Hemoglobinuria / ethnology*
  • Hemoglobinuria / genetics*
  • Hemoglobinuria / metabolism
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Young Adult
  • alpha-Thalassemia / ethnology*
  • alpha-Thalassemia / genetics*
  • alpha-Thalassemia / metabolism
  • beta-Thalassemia / ethnology
  • beta-Thalassemia / genetics
  • beta-Thalassemia / metabolism

Substances

  • Ferritins
  • Hemoglobin H