Clinical heterogeneity of Moroccan patients with hemoglobin C disease

Hemoglobin C disease is an autosomal recessive disorder that results from biparental inheritance of allele encoding hemoglobin C. This mutated form reduces the normal plasticity of host erythrocytes causing a hemoglobinopathy. In homozygotes, nearly all Hemoglobin is in the Hemoglobin C form, resulting in mild hemolytic anemia. The common form of hemoglobin C disease in south Morocco is not usually benign form. His severity and heterogeneity is high. Perhaps, it is due to other local ethnic factors. Here, we studied clinical report, laboratory analysis (by capillary electrophoresis, HPLC and genealogic study) and molecular mechanism in four Moroccan families with hemoglobin C disease. Sepsis, hemostasis and cardiovascular complications can associate to hemoglobin C disease. This genetic disorder can present great health problems. Other factors interact with mutated hemoglobin C, increase oxidative stress and modulate its severity.