RBC Band 3 Protein Reduction in Hereditary Spherocytosis

Also known as: RBC BAND3

Clinical Use

Use

This test is used to confirm a suspected diagnosis of hereditary spherocytosis (HS), a common inherited hemolytic anemia characterized by the presence of spherical erythrocytes (spherocytes). HS is diagnosed based on family history and clinical features, along with clinical laboratory tests such as peripheral smear examination, osmotic fragility (OF), flow cytometry, or by genetic testing. The test measures the reduction of Band 3 protein (solute carrier family 4 member 1, SLC4A1)—the most abundant transmembrane protein in RBCs—by using Eosin-5-maleimide (EMA) dye. The test has a reported sensitivity of 93% for diagnosing HS. Besides HS, positive results may be seen in congenital dyserythropoietic anemia type II, Southeast Asian ovalocytosis, and hereditary pyropoikilocytosis.

Special Instructions

Not provided.

Limitations

The test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. Positive results may also be seen in other rare disorders like congenital dyserythropoietic anemia type II, Southeast Asian ovalocytosis, and hereditary pyropoikilocytosis. The possibility of hereditary spherocytosis cannot be completely ruled out with a normal result, as recent transfusions may affect results.

Test Details

New York Approved