(See also Overview of Hemolytic Anemia
Overview of Hemolytic Anemia
At the end of their normal life span (about 120 days), red blood cells (RBCs) are removed from the circulation. Hemolysis is defined as premature destruction and hence a shortened RBC life span (
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.)
The prevalence of hemoglobin (Hb) C in blacks in the US is about 2 to 3%. Heterozygotes are asymptomatic. Homozyotes usually have a mild chronic hemolytic anemia, splenomegaly, and symptoms consistent with anemia. Cholelithiasis
Cholelithiasis
Cholelithiasis is the presence of one or more calculi (gallstones) in the gallbladder. In developed countries, about 10% of adults and 20% of people > 65 years have gallstones. Gallstones tend...
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is the most common complication, and splenic sequestration is possible.
Hemoglobin C disease is suspected in all patients with a family history and evidence of a hemolytic anemia, particularly in adults with splenomegaly. The anemia is usually mild but can be severe.
The peripheral smear is microcytic, with frequent target cells, spherocytes, and, rarely, crystal-containing red blood cells (RBCs). Nucleated RBCs may be present. The RBCs do not sickle. On electrophoresis, the hemoglobin is type C. In heterozygotes, the only laboratory abnormality is centrally targeted RBCs.
No specific treatment is recommended. Anemia usually is not severe enough to require blood transfusion.
