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Time Sensitive

Ordering Recommendation

Preferred 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.

Specimen Required

Patient Preparation
Collect

Lavender (EDTA), pink (K2EDTA), or green (sodium or lithium heparin). Hemogard tubes are preferred for laboratory automation and safety.

Specimen Preparation

Transport 4 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 stability times provided. Some medication may affect immunophenotyping results and should be provided on the patient test request form.

Stability

EDTA: Ambient: 72 hours; Refrigerated: Unacceptable; Frozen: Unacceptable
Heparin: Ambient: 48 hours; Refrigerated: Unacceptable; Frozen: Unacceptable
New York State Clients: Same as the above.

Reference Interval

Test Number
Components
Reference Interval
  Absolute HLA-DR
Age Reference Interval (cells/µL)
0-6 days 140-2000
1 week-1 month 180-3500
2-4 months 520-2300
5-8 months 130-6300
9-14 months 110-7700
15-23 months 160-3700
2-4 years 180-1300
5-9 years 100-800
10-15 years 120-740
16-64 years 100-640
65 years or older 98-430

  Absolute CD19
Age Reference Interval (cells/µL)
0-6 days 140-2000
1 week-1 month 180-3500
2-4 months 520-2300
5-8 months 130-6300
9-14 months 110-7700
15-23 months 160-3700
2-4 years 180-1300
5-9 years 100-800
10-15 years 120-740
16-64 years 91-610
65 years or older 74-510

  % Natural Killer Cells
Age Reference Interval (Percent)
0-6 days 8-62
1 week-1 month 3-23
2-4 months 2-20
5-8 months 2-36
9-14 months 1-64
15-23 months 1-96
2-4 years 2-25
5-9 years 2-31
10-15 years 4-51
16-64 years 4-26
65 years or older 5-28

  Absolute Natural Killer Cells
Age Reference Interval (cells/µL)
0-6 days 500-3100
1 week-1 month 140-1900
2-4 months 97-2000
5-8 months 68-3900
9-14 months 71-3500
15-23 months 55-4000
2-4 years 61-510
5-9 years 70-590
10-15 years 92-1200
16-64 years 78-470
65 years or older 74-620

  Absolute CD45RO
Age Reference Interval (cells/µL)
0-6 days 98-1300
1 week-1 month 110-1200
2-4 months 90-1400
5-8 months 100-950
9-14 months 160-710
15-23 months 68-630
2-4 years 150-640
5-9 years 100-510
10-15 years 160-700
16-64 years 190-1050
65 years or older 490-1200

  % CD45RO
Age
Reference Interval (Percent)
0-6 days 2-44
1 week-1 month 2-36
2-4 months 1-42
5-8 months 1-46
9-14 months 4-29
15-23 months 5-39
2-4 years 11-50
5-9 years 8-76
10-15 years 18-68
16-64 years 28-72
65 years or older 38-81

  Absolute CD45RA
Age Reference Interval (cells/µL)
0-6 days 900-4500
1 week-1 month 1100-5200
2-4 months 1200-5300
5-8 months 800-5900
9-14 months 900-5200
15-23 months 400-5600
2-4 years 380-2500
5-9 years 250-2000
10-15 years 230-1400
16-64 years 150-870
65 years or older 260-1000

  % CD45RA
Age
Reference Interval (Percent)
0-6 days 60-100
1 week-1 month 63-100
2-4 months 66-100
5-8 months 68-99
9-14 months 68-98
15-23 months 57-100
2-4 years 53-96
5-9 years 47-97
10-15 years 39-93
16-64 years 28-71
65 years or older 19-62

  % HLA-DR
Age Reference Interval (Percent)
0-6 days 3-30
1 week-1 month 3-60
2-4 months 8-33
5-8 months 4-54
9-14 months 3-77
15-23 months 8-45
2-4 years 8-39
5-9 years 4-33
10-15 years 7-24
16-64 years 8-24
65 years or older 7-20

  Absolute CD3
Age Reference Interval (cells/µL)
0-6 days 1400-6800
1 week-1 month 1900-8400
2-4 months 2200-9200
5-8 months 1400-11500
9-14 months 2400-8300
15-23 months 700-8800
2-4 years 850-4300
5-9 years 770-4000
10-15 years 850-3200
16-64 years 570-2400
65 years or older 660-2200

  % CD3
Age Reference Interval (Percent)
0-6 days 38-88
1 week-1 month 55-90
2-4 months 49-97
5-8 months 49-95
9-14 months 56-87
15-23 months 36-92
2-4 years 52-92
5-9 years 55-97
10-15 years 52-90
16-64 years 62-87
65 years or older 62-89

  Absolute CD4
Age
Reference Interval (cells/µL)
0-6 days 1000-4800
1 week-1 month 1500-6000
2-4 months 1600-6500
5-8 months 1000-7200
9-14 months 1300-7100
15-23 months 400-7200
2-4 years 500-2700
5-9 years 400-2500
10-15 years 400-2100
16-64 years 430-1800
65 years or older 490-1600

  Absolute CD8
Age
Reference Interval (cells/µL)
0-6 days 200-2700
1 week-1 month 300-2700
2-4 months 300-3400
5-8 months 200-5400
9-14 months 400-4100
15-23 months 200-2800
2-4 years 200-1800
5-9 years 200-1700
10-15 years 300-1300
16-64 years 210-1200
65 years or older 150-1050

  % CD4
Age Reference Interval (Percent)
0-6 days 26-62
1 week-1 month 39-69
2-4 months 37-69
5-8 months 27-81
9-14 months 25-86
15-23 months 16-91
2-4 years 25-66
5-9 years 26-61
10-15 years 20-65
16-64 years 32-64
65 years or older 35-68

  % CD8
Age Reference Interval (Percent)
0-6 days 5-37
1 week-1 month 7-35
2-4 months 6-41
5-8 months 10-35
9-14 months 7-58
15-23 months 7-40
2-4 years 9-49
5-9 years 13-47
10-15 years 14-40
16-64 years 15-46
65 years or older 10-46

  % CD19
Age Reference Interval (Percent)
0-6 days 3-30
1 week-1 month 3-60
2-4 months 8-33
5-8 months 4-54
9-14 months 3-77
15-23 months 8-45
2-4 years 8-39
5-9 years 4-33
10-15 years 7-24
16-64 years 6-23
65 years or older 5-21

  CD4:CD8 Ratio
Age Reference Interval
0-6 days 1.00-2.60
1 week-1 month 1.30-6.30
2-4 months 1.70-3.90
5-8 months 1.60-3.80
9-14 months 1.30-3.90
15-23 months 0.90-3.70
2-4 years 0.90-2.90
5-9 years 0.90-2.60
10-15 years 0.90-3.40
16-64 years 0.80-3.90
65 years or older 0.80-6.17

  % CD2
Age Reference Interval (Percent)
0-6 days 46-97
1 week-1 month 58-97
2-4 months 51-98
5-8 months 51-98
9-14 months 57-97
15-23 months 37-92
2-4 years 54-92
5-9 years 57-97
10-15 years 56-93
16-64 years 73-91
65 years or older 78-92

  Absolute CD2
Age Reference Interval (cells/µL)
0-6 days 1900-8300
1 week-1 month 2000-9200
2-4 months 2300-10200
5-8 months 1500-13500
9-14 months 2500-10000
15-23 months 750-1080
2-4 years 900-4500
5-9 years 840-4300
10-15 years 950-3800
16-64 years 700-2600
65 years or older 680-2400

Interpretive Data

This profile screens for inherited immunodeficiencies. The CD4 cells are Helper T-cells expressing both CD3 and CD4. The CD8 cells are Cytotoxic T-cells expressing both CD3 and CD8. The B-cells express CD19 but not CD3. The NK-cells express either CD16 or CD56 (or both) but not CD3. CD3, CD4, CD8, CD19 and NK-cell percentages are reported as a percent of total lymphocytes. The CD45RA cells express both CD4 and "naive" CD45RA antigens while CD45RO cells express both CD4 and CD45RO "memory" antigens. CD45RA and CD45RO percentages are reported as a percent of total CD4 cells. 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 agammaglobulinemia) is caused by defective B-cell maturation secondary to mutations in the BTK (Bruton/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 syndrome).

This test was developed and its performance characteristics determined by ARUP Laboratories. It has not been cleared or approved by the US Food and Drug Administration. This test was performed in a CLIA certified laboratory and is intended for clinical purposes.

Compliance Category

Laboratory Developed Test (LDT)

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.

Hotline History

N/A

CPT Codes

86355; 86357; 86359; 86360; 86356 x4

Components

Component Test Code* Component Chart Name LOINC
0095252 Absolute HLA-DR 33617-2
0095615 Lymphocyte Subset Panel 7 Information 48767-8
0095700 Absolute CD19 15195-1
0095701 % Natural Killer Cells 32519-1
0095702 Absolute Natural Killer Cells 20604-5
0095751 Absolute CD45RO 34475-4
0095752 % CD45RO 41994-5
0095816 Absolute CD45RA 26759-1
0095827 % CD45RA 17157-9
0095834 % HLA-DR 31113-4
0095902 Absolute CD3 8122-4
0095903 % CD3 20599-7
0095906 Absolute CD4 24467-3
0095911 Absolute CD8 14135-8
2012857 % CD4 8123-2
2012858 % CD8 32518-3
2012859 % CD19 20593-0
2012860 CD4:CD8 Ratio 54218-3
2012861 % CD2 8118-2
2012862 Absolute CD2 9557-0
* 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
Lymphocyte Subset Panel 7 - Congenital Immunodeficiencies