7.5: Hemoglobin SC Disease

Cause(s):

Both β globin chains are affected as both genes for hemoglobin S and hemoglobin C are both inherited.^1,2

Clinical Features:¹

Complication is less severe than sickle cell disease but more severe than hemoglobin C disease. Cells are still prone to sickling under decreased oxygen tension.

Complications are similar to those seen in sickle cell anemia and vaso-occlusion can occur.

Laboratory Features:^1,2

CBC: Hb: Decreased Hct: Decreased MCHC: Increased	PBS: Normochromic Normocytic Target Cells HbSC crystals	BM: N/A
Hemoglobin Electrophoresis: Hb S: 45% Hb C: 45% Hb A: None Hb A2: 2-4% Hb F: 1%	Other Tests: Solubility Tests: Positive HPLC Hemoglobin Electrophoresis

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References:

1. Laudicina RJ. Hemoglobinopathies: qualitative defects. In: Clinical laboratory hematology. 3rd ed. New Jersey: Pearson; 2015. p.231–50.

2. Randolph TR. Hemoglobinopathies (structural defects in hemoglobin). In: Rodak’s hematology clinical applications and principles. 5th ed. St. Louis, Missouri: Saunders; 2015. p. 426-453.

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