Considered one of the benign hemoglobinopathies, hemoglobin C disease may not be diagnosed until adulthood.
Patients require multispecialty care.
Hemoglobin C is composed of 2 normal alpha chains and 2 variant beta chains, where lysine has replaced glutamic acid at position 6. This unstable hemoglobin precipitates in red cells to form crystals. These intracellular crystals lead to a decrease in red cell deformability and blood that is more viscous. The spleen effectively removes these crystal-containing cells. Some cases may not be diagnosed until adulthood.