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Test Code ALPS Alpha Beta Double-Negative T Cells for Autoimmune Lymphoproliferative Syndrome, Blood

Reporting Name

ALPS Screen

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Useful For

Diagnosing autoimmune lymphoproliferative syndrome, primarily in patients younger than 45 years

Method Name

Flow Cytometry


Shipping Instructions


Testing is performed Monday through Friday. Specimens not received by 4pm (CST) on Friday may be canceled.

 

Collect and package specimen as close to shipping time as possible. It is recommended that specimens arrive within 24 hours of collection.

 

Samples arriving on the weekend and observed holidays may be canceled.



Necessary Information


Ordering healthcare professional name and phone number are required.



Specimen Required


Container/Tube: Lavender top (EDTA)

Specimen Volume: 3 mL

Collection Instructions: Send whole blood specimen in original tube. Do not aliquot.

Additional Information: For serial monitoring, it is recommended that specimens are collected at the same time of day.


Specimen Type

Whole Blood EDTA

Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Whole Blood EDTA Ambient 72 hours PURPLE OR PINK TOP/EDTA

Reject Due To

Gross hemolysis Reject
Gross lipemia Reject

Reference Values

Alpha beta TCR+DNT cells

2-18 years: <2% CD3 T cells

19-70+ years: <3% CD3 T cells

Reference values have not been established for patients that are younger than 24 months of age.

 

Alpha beta TCR+DNT cells

2-18 years: <35 cells/mcL

19-70+ years: <35 cells/mcL

Reference values have not been established for patients that are younger than 24 months of age.

 

Alpha beta TCR+DNT B220+ cells

2-18 years: <0.4% CD3 T cells

19-70+ years: <0.3% CD3 T cells

Reference values have not been established for patients that are younger than 24 months of age.

 

Alpha beta TCR+DNT B220+ cells

2-18 years: <7 cells/mcL

19-70+ years: <6 cells/mcL

Reference values have not been established for patients that are younger than 24 months of age.

 

TCR = T-cell receptor

DNT = Double negative T cell

Interpretation

The presence of increased circulating T cells (CD3+) that are negative for CD4 and CD8 (double-negative T cells: DNT) and positive for the alpha/beta T-cell receptor (TCR) is required for the diagnosis of autoimmune lymphoproliferative syndrome (ALPS).

 

The laboratory finding of increased alpha beta TCR+DNT cells is consistent with ALPS only with the appropriate clinical picture (nonmalignant lymphadenopathy, splenomegaly, and autoimmune cytopenias). Conversely, there are other immunological disorders, including common variable immunodeficiency (CVID), which have subsets for patients with this clinical picture, but no increase in alpha beta TCR+DNT cells.

 

If the percent of the absolute count of either the alpha beta TCR+DNT cells or alpha beta TCR+DNT B220+ cells is abnormal, additional testing is indicated. All abnormal alpha beta TCR+DNT cell results should be confirmed (for ALPS) with additional testing for defective in vitro lymphocyte apoptosis followed by confirmatory genetic testing (ALPSG / Autoimmune Lymphoproliferative Syndrome [ALPS] Gene Panel, Varies).

Day(s) Performed

Monday through Friday

Report Available

3 to 4 days

Test Classification

This test was developed using an analyte specific reagent. Its performance characteristics were determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

86356 x2

86359

LOINC Code Information

Test ID Test Order Name Order LOINC Value
ALPS ALPS Screen 101414-1

 

Result ID Test Result Name Result LOINC Value
23973 %alpha/beta-TCR DNT 34962-1
23974 alpha/beta-TCR DNT 34963-9
28904 % TCR+DNT B220+ 88052-6
28905 Absolute TCR+DNT B220+ 88053-4
23975 Interpretation 69052-9

Forms

If not ordering electronically, complete, print, and send a Benign Hematology Test Request Form (T755) with the specimen.