Autoimmune Lymphoproliferative Profile (INACTIVE as of 08/21/17: Refer to 2014513 in the August Hotline)
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
Frozen or refrigerated specimens. Specimens older than 48 hours. Clotted or hemolyzed specimens. 
Specimens must be analyzed within 48 hours of collection. 
Ambient: 48 hours; Refrigerated: Unacceptable; Frozen: Unacceptable 
Reference Interval
Effective February 16, 2016
Reports include age appropriate reference intervals and interpretation.

Test Number
Age: 0-23 months
Age: 2 years and older
0095516% Alpha/Beta+, CD4-, CD8-0-1.5%0-1.5%
0095517Absolute Alpha/Beta+, CD4-, CD8-0-33 cells/µL0-33 cells/µL
0095518% CD5+, CD20+0-2%0-4%
0095519Absolute CD5+, CD20+0-150 cells/µL0-100 cells/µL
0095521% CD3+, HLA-DR+0-8%0-11%
0095985Absolute CD3+, HLA-DR+0-600 cells/µL0-320 cells/µL

Interpretive Data
In Autoimmune Lymphoproliferative Syndrome (ALPS), all three of the lymphocyte subsets (alpha-beta positive, CD4 negative, and CD8 negative; CD5 positive and CD20 positive; and CD3 positive and HLA-DR positive) are elevated above the reference intervals. CD5 and HLA-DR may be found normally on B-cells and T-cells, respectively, as activation markers. Autoantibody production from CD5 positive B-cells is likely responsible for the autoimmune phenomena seen in ALPS. Increased levels of TCR alpha-beta positive, CD4 negative, and CD8 negative T-cells are the hallmark of ALPS and play a significant role in the pathophysiology of the disease.

Compliance Statement A: Analyte specific reagents (ASR) are used in many laboratory tests necessary for standard medical care and generally do not require U.S. Food and Drug Administration (FDA) approval or clearance. 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, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions. This test should not be regarded as investigational or for research use.

Component Test Code*Component Chart NameLOINC
0095516% Alpha/Beta+, CD4-, CD8-
0095517Absolute Alpha/Beta+, CD4-, CD8-
0095518% CD5+, CD20+8104-2
0095519Absolute CD5+, CD20+33595-0
0095521Absolute CD3+, HLA-DR+26568-6
0095985% CD3+, HLA-DR+18408-5
* 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.
  • Alpha-Beta+, Double Negative (CD4-, CD8-)
  • Alpha/Beta positive
  • ALPS
  • Canale-Smith Syndrome
  • CD4 and CD8 negative
  • DNT
  • Double negative T cells
  • T-Cells for Autoimmune Lymphoproliferative Syndrome (ALPS)