Hemoglobin C: Causes, Symptoms, Diagnosis, Treatment
Last reviewed: 23.04.2024
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[Pathogenesis
The hemoglobin molecule consists of polypeptide chains whose chemical structure is controlled by the corresponding genes. The normal human hemoglobin molecule (HbA) consists of 2 pairs of chains. Blood also normally contains <2.5% HbA (consisting of 5 chains). The level of fetal hemoglobin (HbF containing the y chain instead of f-chains) is gradually decreasing, especially in the first month of life, to <2% of the total hemoglobin of the adult. The concentration of HbF increases with certain violations of hemoglobin synthesis, with aplastic anemia, myelodysplastic syndrome.
Some hemoglobinopathies are manifested by anemia, more pronounced in homozygotes and less in heterozygotes. Some patients are heterozygous for two anomalies and have anemia with characteristic features for both disorders.
Hb is distinguished by electrophoretic mobility and is designated in alphabetical order in accordance with the order of its opening (eg, A, B, C), although the first anomalous hemoglobin was designated as HbS. Structural differences of hemoglobin in accordance with electrophoretic mobility are called over the city or the terrain in which they were discovered (for example, HbS Memphis, HbS Harlem). When describing hemoglobin in a patient having a complex composition, hemoglobin is first to be called, available in a greater concentration.
In the United States, anemia with HbS, HbS and thalassemia are most often detected in immigrants from Southeast Asia, who also have HbE.
Symptoms of the diseases of hemoglobin C
Between 2% and 3% of blacks in the US have an asymptomatic course of the disease. With homozygous form, symptoms are similar to sickle-cell anemia, but less intense. However, abdominal crises, as in sickle-cell anemia, do not occur, and the spleen is usually enlarged. Sequestration of red blood cells in the spleen is possible.
Diagnostics of the diseases of hemoglobin C
Hemoglobin C disease is suspected in all patients with a family history and in blacks with symptomatic sickle cell anemia, especially in adults with splenomegaly. Anemia is usually easily expressed, but can be of moderate severity. As a rule, normocyte anemia with a content of 30 to 100% of target cells, occasionally smear-containing erythrocytes are found in the smear, nucleated erythrocytes can be detected. Crescent erythrocytes are not found. When electrophoresis determines hemoglobin C. With this form of heterozygotes, only target cells are detected.
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Treatment of the diseases of hemoglobin C
Specific treatment is not. Anemia is usually not expressed, and no blood transfusions are required.