Ordering Recommendation

Use to confirm diagnosis of hereditary spherocytosis when hemolytic anemia and spherocytes are present.

New York DOH Approval Status

This test is New York state approved.

Specimen Required

Patient Preparation
Collect

Lavender (EDTA) or green (sodium or lithium heparin). Include a Wright stained slide.

Specimen Preparation

Transport 4 mL whole blood in the original container. (Min: 0.5 mL)

Storage/Transport Temperature

Refrigerated.

Unacceptable Conditions

Clotted or hemolyzed specimens. Specimens older than 7 days.

Remarks

Specimens must be analyzed within 7 days of collection.

Stability

Ambient: 3 days; Refrigerated: 7 days; Frozen: Unacceptable

Methodology

Qualitative Flow Cytometry

Performed

Sun-Sat

Reported

1-3 days

Reference Interval

Normal

Interpretive Data

This test can be used to confirm a suspected diagnosis of hereditary spherocytosis (HS). HS is 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, including peripheral smear examination, osmotic fragility (OF), flow cytometry, or by genetic testing (Hereditary Hemolytic Anemia Panel Sequencing, ARUP test code 2012052).

Band 3 (or solute carrier family 4 member 1, SLC4A1) is the most abundant transmembrane protein found in human red blood cells (RBC). Eosin-5-maleimide (EMA) dye binds to band 3 on intact RBC's. A reduction of fluorescence intensity will be seen in hereditary spherocytosis. This test by flow cytometry has been reported to have a sensitivity of 93 percent for a diagnosis of HS. Congenital dyserythropoietic anemia type II, Southeast Asian ovalocytosis and hereditary pyropoikilocytosis are rare disorders that may also show a positive result.

Compliance Category

Laboratory Developed Test (LDT)

Note

Hotline History

N/A

CPT Codes

88184

Components

Component Test Code* Component Chart Name LOINC
2008461 RBC Band 3 Protein Reduction in HS 33048-0
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Aliases

  • Hereditary Spherocytosis, EMA, BAND 3, Osmotic Fragility
  • HS hemolytic anema assay
RBC Band 3 Protein Reduction in Hereditary Spherocytosis