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Overall, hemoglobin C disease is one of the more benign hemoglobinopathies. [6] Mild-to-moderate reduction in RBC lifespan may accompany from mild hemolytic anemia. Individuals with hemoglobin C disease have sporadic episodes of musculoskeletal (joint) pain. [6] People with hemoglobin C disease can expect to lead a normal life. [14]
The mutation which causes sickle cell disease results in an abnormal hemoglobin known as hemoglobin S (HbS), which replaces HbA in adults. [19] The human genome contains a pair of genes for β-globin; in people with sickle cell disease, both genes are affected and the erythropoietic cells in the bone marrow will only create HbS.
Hemoglobin S/ beta thalassemia: (see above). [18] Hemoglobin S/ hemoglobin C (Hemoglobin SC disease) occurs when an individual inherits one gene for hemoglobin S (sickle cell) and one gene for hemoglobin C, The symptoms are very similar to sickle cell disease. [19]
Some findings also show the reduction of the sickle-cell trait in those who retain much more fetal hemoglobin than usual in adulthood. Fetal hemoglobin likely plays a role in the prevention of sickling. Elevated fetal hemoglobin levels have been observed in populations where sickle-cell disease is prevalent. [20] [5] [21]
Hemoglobin electrophoresis is a blood test that can detect different types of hemoglobin. The test can detect hemoglobin S, the form associated with sickle cell disease, as well as other abnormal types of hemoglobin, such as hemoglobin C. It can also be used to investigate thalassemias, which are disorders caused by defective hemoglobin production.
"Sickle Cell Anemia, a Molecular Disease" is a 1949 scientific paper by Linus Pauling, Harvey A. Itano, Seymour J. Singer and Ibert C. Wells that established sickle-cell anemia as a genetic disease in which affected individuals have a different form of the metalloprotein hemoglobin in their blood.
Anti-Kell hemolytic disease of the newborn; Rhesus c hemolytic disease of the newborn; Rhesus E hemolytic disease of the newborn; Other blood group incompatibility (RhC, Rhe, Kid, Duffy, MN, P and others) Drug induced immune mediated hemolytic anemia Penicillin (high dose) Methyldopa; Hemoglobinopathies (where these is an unstable or ...
Treatment is the same as for patients with sickle cell disease. Patients may receive hydroxyurea to induce the protective effects of increased fetal hemoglobin production. They may also benefit from blood transfusions especially during vaso-occlusive crises. Patients may be offered chemoprophylaxis with penicillin.