7.5: Hemoglobin SC Disease

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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

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.