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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
ARUP Consult®
Disease Topics
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: EDTA:
Ambient: 30 hours; Refrigerated: Unacceptable; Frozen: Unacceptable
Heparin:
Ambient: 48 hours; Refrigerated: Unacceptable; Frozen: Unacceptable  
Reference Interval
Effective November 16, 2015

Reports include age appropriate reference intervals and interpretation.

Reference Interval Notes:
Pediatric reference values (0 - 6 days up to 10 - 15 years) taken from Scandinavian Journal of Immunology 2012; 75, 436-444.
Adult and Geriatric (16 - 64 and 65 plus years) ranges were developed in-lab.
Publications did not address HLA-DR
; CD19 ranges were used for all HLA-DR pediatric ranges (0 - 6 days up to 10 - 15 years).
Test Number
Components
0-6 days
1 week-1 month
2-4 months
5-8 months
9-14 months
15-23 months
2-4 years
5-9 years
10-15 years
16-64 years
65 years or older
% CD246-97%58-97%51-98%51-98%57-97%37-92%54-92%57-97%56-93%73-91%78-92%
Absolute CD21900-8300 cells/µL2000-9200 cells/µL2300-10200 cells/µL1500-13500 cells/µL2500-10000 cells/µL750-10800 cells/µL900-4500 cells/µL840-4300 cells/µL950-3800 cells/µL700-2600 cells/µL680-2400
cells/µL
% CD338-88%55-90 %49-97 %49-95%56-87%36-92%52-92%55-97%52-90%62-87%62-89%
Absolute
CD3
1400-6800 cells/µL1900-8400 cells/µL2200-9200 cells/µL1400-11500 cells/µL2400-8300 cells/µL700-8800 cells/µL850-4300 cells/µL770-4000 cells/µL850-3200 cells/µL570-2400 cells/µL660-2200 cells/µL
% HLA-DR3-30%3-60%8-33%4-54%3-77%8-45%8-39%4-33%7-24%8-24%7-20%
Absolute
HLA-DR
140-2000 cells/µL180-3500 cells/µL520-2300 cells/µL130-6300 cells/µL110-7700 cells/µL160-3700 cells/µL180-1300 cells/µL100-800 cells/µL120-740 cells/µL100-640 cells/µL98-430 cells/µL
% CD426-62 %39-69 %37-69 %27-81%25-86%16-91%25-66%26-61%20-65%32-64%35-68%
Absolute
CD4
1000-4800 cells/µL1500-6000 cells/µL1600-6500 cells/µL1000-7200 cells/µL1300-7100
cells/µL
400-7200 cells/µL500-2700 cells/µL400-2500 cells/µL400-2100 cells/µL430-1800 cells/µL490-1600 cells/µL
% CD45RA60-100%63-100%66-100%68-99%68-98%57-100%53-96%47-97%39-93%28-71%19-62%
Absolute
CD45RA
900-4500 cells/µL1100-5200 cells/µL1200-5300 cells/µL800-5900 cells/µL900-5200 cells/µL400-5600 cells/µL380-2500 cells/µL250-2000 cells/µL230-1400 cells/µL350-1100 cells/µL260-1000 cells/µL
% CD45RO2-44%2-36%1-42%1-46%4-29%5-39%11-50%8-76%18-68%28-72%38-81%
Absolute
CD45RO
98-1300 cells/µL110-1200 cells/µL90-1400 cells/µL100-950 cells/µL160-710 cells/µL68-630 cells/µL150-640 cells/µL100-510 cells/µL160-700 cells/µL340-1150 cells/µL490-1200 cells/µL
% CD85-37%7-35%6-41%10-35%7-58%7-40%9-49%13-47%14-40%15-46%10-46%
Absolute
CD8
200-2700 cells/µL300-2700
cells/µL
300-3400 cells/µL200-5400 cells/µL400-4100 cells/µL200-2800 cells/µL200-1800 cells/µL200-1700 cells/µL300-1300 cells/µL210-1200 cells/µL150-1050 cells/µL
CD4:CD8 Ratio1.00-2.601.30-6.301.70-3.901.60-3.801.30-3.900.90-3.700.90-2.900.90-2.600.90-3.400.80-3.900.80-6.17
% CD193-30%3-60%8-33%4-54%3-77%8-45%8-39%4-33%7-24%6-23%5-21%
Absolute CD19140-2000 cells/µL180-3500 cells/µL520-2300 cells/µL130-6300 cells/µL110-7700 cells/µL160-3700 cells/µL180-1300 cells/µL100-800 cells/µL120-740 cells/µL91-610 cells/µL74-510 cells/µL
% NK-cells8-62%3-23%2-20%2-36%1-64%1-96%2-25%2-31%4-51%4-26%5-28%
Absolute
NK-cells
500-3100 cells/µL140-1900 cells/µL97-2000 cells/µL68-3900 cells/µL71-3500 cells/µL55-4000 cells/µL61-510 cells/µL70-590 cells/µL92-1200 cells/µL78-470 cells/µL74-620 cells/µL

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).


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.
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 x5
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
0095906Absolute CD424467-3
0095911Absolute CD814135-8
2012857% CD48123-2
2012858% CD832518-3
2012859% CD1920593-0
2012860CD4:CD8 Ratio54218-3
2012861% CD220594-8
2012862Absolute 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