Alpha/Beta Double-Negative T-Cells for Autoimmune Lymphoproliferative Syndrome
Ordering Recommendation

Support the diagnosis of autoimmune lymphoproliferative syndrome (ALPS).

Quantitative Flow Cytometry
1-3 days
New York DOH Approval Status
Specimens from New York clients will be sent out to a New York DOH approved laboratory, if possible.
Submit With Order
ARUP Consult®
Disease Topics
Specimen Required
Patient Preparation
Green (Sodium or Lithium Heparin), Lavender (EDTA), or Pink (K2EDTA) 
Specimen Preparation
Transport 5 mL whole blood. (Min: 0.5 mL) 
Storage/Transport Temperature
Unacceptable Conditions
Clotted or hemolyzed specimens 
Specimens must be analyzed within 48 hours of collection 
Ambient: 48 hours; Refrigerated: Unacceptable; Frozen: Unacceptable 
Reference Interval
Reports include age appropriate reference intervals and interpretation.
Test NumberComponentsAge: 2-18 years oldAge 18-69 years old
Absolute alpha/beta TCR+ DNT0-46 (cells/uL)0-32 (cells/uL)
Absolute alpha/beta TCR+ DNT B220+0-5 (cells/uL)0-6 (cells/uL)
% alpha/beta TCR+ DNT0-3 (%)0-2 (%)
% alpha/beta TCR+ DNT B220+0-0.3 (%)0-0.4 (%)

Interpretive Data
Reported percentages reflect percentage of CD3 positive lymphocytes. The hallmark for a diagnosis of Autoimmune Lymphoproliferative Syndrome (ALPS) is an increased concentration of CD3+ T-cells 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 a sensitive and specific marker for ALPS and is associated with mutations in the FAS gene.

Abnormal results should be correlated with clinical history and confirmed by additional testing for defective in vitro lymphocyte apoptosis and for mutations in the FAS gene.

Statement A: Compliance Statement A: For laboratory developed tests using a manufacturer labeled ASR as the reagent providing the specificity of the assay. Analyte Specific Reagent. This test was developed and its performance characteristics determined by ARUP Laboratories. The U. S. Food and Drug Administration has not approved or cleared this test; however, the FDA has determined that such clearance or approval is not necessary.

Hotline History
View Hotline History
Component Test Code*Component Chart NameLOINC
2014514Absolute alpha/beta TCR+ DNT
2014515Absolute alpha/beta TCR+ DNT B220+
2014516% alpha/beta TCR+ DNT
2014517% alpha/beta TCR+ DNT B220+
* Component test codes cannot be used to order tests. The information provided here is not sufficient for interface builds; for a complete test mix, please view this test within the Laboratory Test Directory found at