Alpha Beta Double Negative T-Cells for Autoimmune Lymphoproliferative Syndrome
Also known as: ALPS ABDNT
Use
This test aids in the diagnosis of autoimmune lymphoproliferative syndrome (ALPS), which is characterized by an increased concentration of CD3+ T-cells that are negative for CD4 and CD8 (double-negative T-cells [DNT]) and positive for the alpha/beta T-cell receptor (TCR). B220 expression on alpha/beta TCR+DNT cells is used as a sensitive and specific marker for ALPS, which is often associated with mutations in the FAS gene. Abnormal results should be correlated with clinical history and confirmed by additional testing.
Special Instructions
Not provided.
Limitations
The test is not approved by the US Food and Drug Administration; it was developed and its performance characteristics were determined by ARUP Laboratories. This test is intended for clinical purposes and is performed in a CLIA-certified laboratory. The results must be interpreted in the context of the patient's clinical history and correlated with other testing for mutations in the FAS gene and defective in vitro lymphocyte apoptosis.
Methodology
Cell-based / Cytometry (Flow Cytometry)
Biomarkers
CD3+ T-cells negative for CD4 and CD8, positive for alpha/beta TCR
Cell Type
LOINC Codes
- 34963-9 - CD3+TCR A-B+ Cells # Bld
- 88053-4 - Cell.DNT-CD45R+TCRa b # Bld FC
- 34962-1 - CD3+TCR A-B+ Cells NFr Bld
Result Turnaround Time
1-3 days
Related Documents
For more information, please review the documents below
Specimen
Whole Blood
Volume
5 mL
Minimum Volume
0.5 mL
Container
Green (sodium or lithium heparin), lavender (EDTA), or pink (K2EDTA)
Storage Instructions
CRITICAL ROOM TEMPERATURE
Causes for Rejection
Clotted or hemolyzed specimens
Stability Requirements
| Temperature | Period |
|---|---|
| Room Temperature | 48 hours |
| Refrigerated | Unacceptable |
| Frozen | Unacceptable |
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