Icon

Time Sensitive
Ordering Recommendation

Support the diagnosis of autoimmune lymphoproliferative syndrome (ALPS).

Mnemonic
ALPS ABDNT
Methodology

Quantitative Flow Cytometry

Performed

Sun-Sat

Reported

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.
Specimen Required
Patient Preparation
Collect

Green (Sodium or Lithium Heparin), Lavender (EDTA), or Pink (K2EDTA)

Specimen Preparation

Transport 5 mL whole blood. (Min: 0.5 mL)

Storage/Transport Temperature

CRITICAL ROOM TEMPERATURE

Unacceptable Conditions

Clotted or hemolyzed specimens

Remarks

Specimens must be analyzed within 48 hours of collection

Stability

Ambient: 48 hours; Refrigerated: Unacceptable; Frozen: Unacceptable

Reference Interval

Reports include age appropriate reference intervals and interpretation.

Test Number Components Age: 2-18 years old Age 18-69 years old
Absolute alpha/beta TCR+ DNT 0-46 (cells/uL) 0-32 (cells/uL)
Absolute alpha/beta TCR+ DNT B220+ 0-5 (cells/uL) 0-6 (cells/uL)
% alpha/beta TCR+ DNT 0-3 (%) 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.

Compliance Category

Laboratory Developed Test (LDT)

Note
Hotline History
N/A
CPT Codes

86356 x2

Components
Component Test Code* Component Chart Name LOINC
2014514 Absolute alpha/beta TCR+ DNT 34963-9
2014515 Absolute alpha/beta TCR+ DNT B220+ 88053-4
2014516 % alpha/beta TCR+ DNT 34962-1
2014517 % alpha/beta TCR+ DNT B220+ 88053-4
* 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 click the sidebar link to access the Interface Map.
Aliases
Alpha/Beta Double-Negative T-Cells for Autoimmune Lymphoproliferative Syndrome