Lymphocyte Subset Panel 7 - Congenital Immunodeficiencies
0095899
Ordering Recommendation
Acceptable lymphocyte subset panel for the investigation of primary immunodeficiency disorders.
Mnemonic
PIP
Methodology
Quantitative Flow Cytometry
Performed
Sun-Sat
Reported
1-3 days  
New York DOH Approval Status
This test is New York DOH approved.
Submit With Order
Specimen Required
Patient Preparation
  
Collect
Lavender Hemogard (EDTA), pink Hemogard (K2EDTA), or green Hemogard (sodium or lithium heparin). Hemogard tubes are preferred for laboratory safety.  
Specimen Preparation
Transport 4 mL whole blood. (Min: 0.5 mL)  
Storage/Transport Temperature
CRITICAL ROOM TEMPERATURE.  
Unacceptable Conditions
Frozen or refrigerated specimens. Specimens older than 72 hours in EDTA or 48 hours in heparin. Clotted or hemolyzed specimens.
New York State Clients:
 Specimens collected in heparin. Frozen or refrigerated specimens. EDTA specimens older than 30 hours. Clotted or hemolyzed specimens.  
Remarks
Specimens must be analyzed within 72 hours of collection in EDTA and within 48 hours of collection in heparin. Some medications may affect immunophenotyping results and should be listed on the patient test request form.
New York State Clients:
Only EDTA specimens may be submitted and must be analyzed within 30 hours of collection.  
Stability
Ambient: 72 hours in EDTA, 48 hours in heparin; Refrigerated: Unacceptable; Frozen: Unacceptable
New York State Clients:
EDTA: Ambient: 30 hours, Refrigerated: Unacceptable; Frozen: Unacceptable  
Reference Interval
Reports include age appropriate reference intervals and interpretation.
 
Components Age: 0-​11 months Age: 12-​23 months Age: 2 years and older
% CD2 55-​88 % 55-​88 % 73-​91 %
Absolute CD2 3800-​5300 cells/µL 3100-​4200 cells/µL 700-​2600 cells/µL
% CD3 58-​85 % 53-​81 % 62-​87 %
Absolute CD3 2170-​6500 cells/µL 1460-​5440 cells/µL 570-​2400 cells/µL
% HLA-​DR 11-​45 % 11-​45 % 8-​24 %
Absolute HLA-​DR 430-​3300 cells/µL 430-​3300 cells/µL 100-​640 cells/µL
% CD4 38-​62 % 31-​54 % 32-​64 %
Absolute CD4 1580-​4850 cells/µL 1020-​3600 cells/µL 430-​1800 cells/µL
% CD45RA 15-​70 % 15-​70 % 5-​37 %
Absolute CD45RA 200-​3400 cells/µL 200-​3400 cells/µL 130-​1100 cells/µL
% CD45RO 5-​30 % 5-​30 % 12-​38 %
Absolute CD45RO 50-​1500 cells/µL 50-​1500 cells/µL 220-​1000 cells/µL
% CD8 16-​34 % 16-​38 % 15-​46 %
Absolute CD8 680-​2470 cells/µL 570-​2230 cells/µL 210-​1200 cells/µL
CD4:CD8 ratio 1.17-​6.62 1.17-​6.62 0.80-​3.90
% CD19 11-​45 % 11-​45 % 6-​23 %
Absolute CD19 430-​3300 cells/µL 430-​3300 cells/µL 91-​610 cells/µL
% NK-​cells 3-​19 % 3-​19 % 4-​26 %
Absolute NK-​cells 80-​340 cells/µL 80-​340 cells/µL 78-​470 cells/µL
Interpretive Data
This profile screens for inherited immunodeficiencies. T-cell subsets (include naive and memory T-cells), B-cells, and NK-cells are evaluated. Primary immune deficiencies that show phenotypic abnormalities include X-linked hypogammaglobulinemia, DiGeorge syndrome, bare lymphocyte syndrome, and severe combined immunodeficiency disease (SCID).

X-linked hypogammaglobulinemia (X-linked agammaglobulinemia, Bruton's agammaglobulinemia) is caused by defective B-cell maturation secondary to mutations in the btk (Bruton's/B-cell tyrosine kinase) gene. T-cells (CD2, CD3) are normal or increased in number, and the CD4:CD8 ratio is normal or decreased. Most of the CD4 cells express the CD45RA antigen characteristic of naive rather than memory cells. B-cells (CD19, HLA-DR) are severely decreased or absent in the peripheral blood.

X-linked hypogammaglobulinemia can be distinguished from transient hypogammaglobulinemia of infancy by the absence of B-cells. Transient hypogammaglobulinemia of infancy results from delayed capacity for immunoglobulin synthesis and spontaneously resolves with age.

Thymic aplasia (congenital thymic aplasia, DiGeorge syndrome) results in impaired T-cell maturation and function. B-cells (CD19, HLA-DR) and NK-cells (CD16/CD56) are normal but T-cells (CD2, CD3) are usually decreased with an elevated CD4:CD8 ratio. The clinical course is variable, ranging from "partial DiGeorge syndrome" to cases that resemble SCID.

SCID has multiple genetic causes, including mutations in the gamma chain of the interleukin 2 receptor and the purine degradation enzymes, adenosine deaminase, and nucleoside phosphorylase. In adenosine deaminase deficiency, both B-cells (CD19, HLA-DR) and T-cells (CD2, CD3) are decreased in the peripheral blood. In other forms of SCID, the lymphopenia is not as severe, but the lymphocyte count is usually less than 1,000/µL even though B-cells (CD19, HLA-DR) may be normal or increased. In contrast to thymic aplasia, any T-cells present may have an immature phenotype.

Major histocompatibility complex class II deficiency, bare lymphocyte syndrome, is caused by defective transcription of HLA class II genes; B-cells (CD19) and T-cells (CD2, CD3) are present in normal numbers, but HLA-DR is absent. The CD4+ cells are usually CD45RA+.

Common variable immunodeficiency (CVID) describes a heterogeneous group of disorders with defective antibody formation. B-cells (CD19, HLA-DR) and T-cells (CD2, CD3) are usually normal in number, although B-cells may be decreased when CVID occurs concurrently with systemic lupus erythematosus. The CD4:CD8 ratio may be normal or decreased.

Wiskott-Aldrich syndrome includes immunodeficiency with thrombocytopenia and eczema. Lymphopenia is usually present with a progressive decline in T-cells numbers. The CD4:CD8 ratio is normal. The gene is X-linked and encodes the Wiskott-Aldrich syndrome protein.

Immunophenotyping is generally not useful in characterizing selective IgA deficiency, IgG subclass deficiencies, the hyper IgM syndrome, or hyperimmunoglobulin E syndrome (Job's syndrome).

See Compliance Statement A: www.aruplab.com/CS
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.
 
Note
This assay is designed for enumerating the percents and absolute cell counts of lymphocyte subsets in lysed whole blood. Whole blood is added to fluorochrome-labeled antibodies that bind specifically to cell surface antigens on lymphocytes. After incubation, lysing, and fixation, percents and absolute counts are enumerated utilizing an internal quantitation standard. Additional CBC data is not required.
CPT Code(s)
86355; 86357; 86359; 86360; 86356 x4
Components
Component Test Code*Component Chart NameLOINC
0095252Absolute HLA-DR33617-2
0095615Lymphocyte Subset Panel 7 Information48767-8
0095700Absolute CD1915195-1
0095701% Natural Killer Cells32519-1
0095702Absolute Natural Killer Cells20604-5
0095751Absolute CD45RO26570-2
0095752% CD45RO26573-6
0095816Absolute CD45RA26759-1
0095827% CD45RA17157-9
0095834% HLA-DR32751-0
0095902Absolute CD38122-4
0095903% CD320599-7
0095905% CD48123-2
0095906Absolute CD424467-3
0095910% CD832518-3
0095911Absolute CD814135-8
0095914% CD1920593-0
0095920CD4:CD8 Ratio54218-3
0095930% CD220594-8
0095931Absolute CD29557-0
* 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
  • Primary Immunodeficiency Profile