NIH Clinical Center
Dept. of Lab Medicine | Dept. of Transfusion Medicine | Anatomic Pathology Test Guide > Lab Test
 Lab Test
 
 Test Description
Panel Name:
Test Name:
Beta-2 Glycoprotein 1 Antibody Evaluation
Panel Tests:
LIS Test Code:B2GI2
Synonyms: B2GPI, B2GP1
Department:Laboratory Medicine
Lab Area:Mayo Medical Labs
Telephone:301-496-5972
TAT:2-6 days
 Specimen Collection
Source:

Site:

Container:Red/Yellow Rim 4mL SST

Micro. Container:


Collection Instructions:



Delivery Instructions:Send specimen to 2C324 or Tube Station 321
Specimen Type: Blood
Frequency / Restrictions:
 Expected Values/Results

Ranges and Interpretation:

Beta-2 Glycoprotein 1 Antibody Evaluation
Performed at Mayo Medical Labs, Rochester MN
Effective Effective 06Mar07 – present:
Reference Values:
Negative:             <10 U/mL
Borderline: 10.0 - 14.9 U/mL
Positive:         >= 15.0 U/mL

Interpretation:

Strongly positive results for Beta 2 GP1 antibodies (>40 U/mL for IgG and/or IgM) are diagnostic criterion for APS. Lesser levels of Beta 2 GP1 antibodies and antibodies of the IgA isotype may occur in patients with clinical signs of APS, but the results are not considered diagnostic. Beta 2 GP1 antibodies must be detected on 2 or more occasions at least 12 weeks apart to fulfill the laboratory diagnostic criteria for APS.

Detection of Beta 2 GP1 antibodies is not affected by anticoagulant treatment.


Organisms Reported:

Alert Limits:

 Additional Test Information

Comments:

Beta 2 glycoprotein 1 (Beta 2 GP1, also called apolipoprotein H) is a 326-amino acid polypeptide synthesized by hepatocytes, endothelial cells, and trophoblast cells. It contains 5 homologous domains of approximately 60 amino acids each.(1,2) Domain 5, located at the C terminus, contains a hydrophobic core surrounded by 14 positively charged amino acid residues that promote electrostatic interactions with plasma membranes via interactions with negatively charged phospholipids. Complexes of Beta 2 GP1 and phospholipids in vivo reveal epitopes that react with natural autoantibodies.(3) Plasma from normal individuals contains low concentrations of IgG autoantibodies to Beta 2 GP1 (Beta 2 GP1 antibodies) that are of moderate affinity and react with an epitope on the first domain near the N terminus.

Pathologic levels of Beta 2 GP1 antibodies occur in patients with antiphospholipid syndrome (APS). APS is associated with a variety of clinical symptoms, notably, thrombosis, pregnancy complications, unexplained cutaneous circulatory disturbances (livido reticularis or pyoderma gangrenosum), thrombocytopenia or hemolytic anemia, and nonbacterial thrombotic endocarditis. Beta 2 GP1 antibodies are found with increased frequency in patients with systemic rheumatic diseases, especially systemic lupus erythematosus.

Beta 2 GP1 antibodies are detected in the clinical laboratory by different types of assays including immunoassays and functional coagulation assays. Immunoassays for Beta 2 GP1 antibodies can be performed using either a composite substrate comprised of Beta 2 GP1 plus anionic phospholipid (eg, cardiolipin or phosphatidylserine), or Beta 2 GP1 alone. Antibodies detected by immunoassays that utilize composite substrates are commonly referred to as phospholipid or cardiolipin antibodies. Antibodies detected using Beta 2 GP1 substrate without phospholipid (so-called direct assays) are referred to simply as Beta 2 GP1 antibodies. Some Beta 2 GP1 antibodies are capable of inhibiting clot formation in functional coagulation assays that contain low concentrations of phospholipid cofactors. Antibodies detected by functional coagulation assays are commonly referred to as lupus anticoagulants.

The diagnosis of APS requires at least 1 clinical criteria and 1 laboratory criteria be met.(5) The clinical criteria include vascular thrombosis (arterial or venous in any organ or tissue) and pregnancy morbidity (unexplained fetal death, premature birth, severe preeclampsia, or placental insufficiency). Other clinical manifestations, including heart valve disease, livedo reticularis, thrombocytopenia, nephropathy, neurological symptoms, are often associated with APS but are not included in the diagnostic criteria. The laboratory criteria for diagnosis of APS are presence of lupus anticoagulant, presence of IgG and/or IgM anticardiolipin antibody (>40 GPL, >40 MPL, or >99th percentile), and/or presence of IgG and/or IgM Beta 2 GP1 antibody (>99th percentile). All antibodies must be demonstrated on 2 or more occasions separated by at least 12 weeks. Direct assays for Beta 2 GP1 antibodies have been reported to be somewhat more specific (but less sensitive) for disease diagnosis in patients with APS.(4) Anticardiolipin and Beta 2 GP1 antibodies of the IgA isotype are not part of the laboratory criteria for APS due to lack of specificity.

CLINICAL REFERENCE:

1. Caronti B, Calderaro C, Alessandri C, et al: Beta 2 glycoprotein 1 (beta-2 GP1) mRNA is expressed by several cell types involved in anti-phospholipid syndrome-related tissue damage. Clin Exp Immunol 1999;115:214-219

2. Lozier J, Takahashi N, Putnam F: Complete amino acid sequence of human plasma beta 2 glycoprotein 1. Proc Natl Acad Sci USA 1984;81:3640-3644

3. Kra-Oz Z, Lorber M, Shoenfeld Y, Scharff Y: Inhibitor(s) of natural anti-cardiolipin autoantibodies. Clin Exp Immunol 1993;93:265-268

4. Audrain MA, El-Kouri D, Hamidou MA, et al: Value of autoantibodies to beta 2 glycoprotein 1 in the diagnosis of antiphospholipid syndrome. Rheumatology (Oxford) 2002;41:550-553

5. Wilson WW, Gharavi AE, Koike T, et al: International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Arthritis Rheum 1999;42(7):1309-1311



Methodology:

Enzyme Linked Immunosorbent Assay (ELISA)


Cautions:

The immunoassay for Beta 2 GP1 antibodies does not distinguish between autoantibodies and antibodies produced in response to infectious agents or as epiphenomena following thrombosis. For this reason, a single positive test result is not sufficient to meet accepted serologic criteria for the diagnosis of APS (see above).

Comparative studies and interlaboratory proficiency surveys indicate that results of Beta 2 GP1 antibody tests can be highly variable and results obtained with different commercial immunoassays may yield substantially different results.(5)

 
Archived Ranges:Historical Reference Ranges



 
 

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