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Hemoglobin S-C disease: causes, symptoms, diagnosis, treatment

 
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Last reviewed: 23.04.2024
 
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Hemoglobin S-C disease is hemoglobinopathy, the symptom of which is similar to that of sickle-cell anemia, but less intense.

Since 10% of Negroid people have an abnormality of HbS, the heterozygous combination of S-C occurs more often than the homozygous form of HbC. Anemia in abnormal HbS-C is similar to the HbC anomaly, but is more easily expressed; some patients even have a normal level of hemoglobin. Most of the symptoms of the disease are similar to sickle-cell anemia, but less intense and less common. However, typical signs are marked hematuria, hemorrhages in the retina of the eye, aseptic necrosis of the head of the femur. HbS-S disease is suspected in all patients with symptoms of sickle-cell anemia or in the detection of crescent morphology of erythrocytes. In stained smears, there are visible and occasionally crescent-shaped cells. Sickling is identified in special preparations, and electrophoresis is used to confirm the diagnosis.

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