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Time Sensitive

Ordering Recommendation

Support the diagnosis of autoimmune lymphoproliferative syndrome (ALPS).

New York DOH Approval Status

Testing is not New York state approved. Specimens from New York clients will be sent out to a New York state-approved laboratory.

Specimen Required

Patient Preparation
Collect

Green (sodium or lithium heparin), lavender (EDTA), or pink (K2EDTA)
New York State Clients: Lavender (EDTA) or pink (K2EDTA)

Specimen Preparation

Transport 5 mL whole blood. (Min: 0.5 mL)
New York State Clients: Specimen must be received at performing laboratory within 72 hours. Do not send to ARUP Laboratories. For direct submission instructions please contact ARUP Referral Testing at 800-242-2787 ext. 5161.

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
New York State Clients: Ambient: 72 hours; Refrigerated: Unacceptable; Frozen: Unacceptable

Methodology

Quantitative Flow Cytometry

Performed

Sun-Sat

Reported

1-3 days

Reference Interval

Test Number
Components
Reference Interval
  Absolute alpha/beta TCR+ DNT
Age
Reference Interval (cells/uL)
2-18 years old 0-46
18-69 years old 0-32

  Absolute alpha/beta TCR+ DNT B220+
Age
Reference Interval (cells/uL)
2-18 years old 0-5
18-69 years old 0-6

  % alpha/beta TCR+ DNT
Age
Reference Interval (%)
2-18 years old 0-3
18-69 years old 0-2

  % alpha/beta TCR+ DNT B220+
Age
Reference Interval (%)
2-18 years old 0-0.3
18-69 years old 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