Sign in →

Test Code 13ACT Coagulation Factor XIII (13) Activity Assay, Plasma


Ordering Guidance


Coagulation testing is highly complex, often requiring the performance and correlation of factor XIII activity with multiple assays and correlation with clinical information. For that reason, if indicated, consider ordering ALBLD / Bleeding Diathesis Profile, Limited, Plasma.



Specimen Required


Patient Preparation:

1. Fasting: 8 hours, preferred but not required

2. It is best to perform this study pre-plasma transfusion if possible. If patient has been recently transfused, wait at least 48 hours after transfusion before collecting the specimen.

3. Specimen should be collected prior to factor replacement therapy.

Collection Container/Tube: Light-blue top (3.2% sodium citrate)

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL Platelet-poor plasma

Collection Instructions:

1. For complete instructions, see Coagulation Guidelines for Specimen Handling and Processing.

2. Centrifuge, transfer all plasma into a plastic vial, and centrifuge plasma again.

3. Aliquot plasma into a plastic vial, leaving 0.25 mL in the bottom of centrifuged vial.

4. Freeze plasma immediately (no longer than 4 hours after collection) at -20° C or, ideally, -40° C or below.

Additional Information:

1. Double-centrifuged specimen is critical for accurate results.

2. Each coagulation assay requested should have its own vial.


Useful For

Measuring factor XIII activity

Method Name

Fluorescence-Based Enzyme Activity

Reporting Name

Factor XIII(13) Activity, P

Specimen Type

Plasma Na Cit

Specimen Minimum Volume

Platelet-poor plasma: 0.5 mL

Specimen Stability Information

Specimen Type Temperature Time
Plasma Na Cit Frozen 180 days

Reject Due To

Gross Hemolysis Reject
Gross Lipemia Reject
Gross Icterus Reject

Clinical Information

Factor XIII (FXIII) is found in plasma and platelets. Plasma FXIII consists of 2 A subunits and 2 B subunits; platelet FXIII consists of only 2 A subunits. After FXIII is activated by thrombin, it catalyzes the formation of peptide bonds between adjacent molecules of fibrin monomers, thus conferring mechanical and chemical stability to the fibrin clot. Fibrin that is not covalently crosslinked exhibits an increased susceptibility to fibrinolysis.

 

Congenital FXIII deficiency is an autosomal recessive bleeding disorder. Individuals with severe FXIII deficiency (FXIII:C <1%) may experience intracranial hemorrhage, soft tissue hemorrhage, hemarthrosis, and hematomas. Typically, affected patients suffer from delayed bleeding occurring 24 to 48 hours after the initial hemostatic response to an injury. In newborns, bleeding from the umbilical stump may occur after separation of the umbilical cord, as well as intracranial bleeding. Poor wound healing and abnormal scar formation are also observed. Asymptomatic individuals with milder deficiencies may experience post-surgical or trauma-related bleeding; additionally female patients may experience recurrent spontaneous abortions and heavy menstrual bleeding.

 

Although felt to be rare, acquired FXIII deficiency is likely more commonly encountered than congenital deficiency state. In one series of patients, the large majority of patients had an acquired deficiency state as opposed to a congenital deficiency. The various etiologies included consumptive coagulopathy, infection related, or due to development of autoantibodies. These patients develop adult-onset bleeding.

Reference Values

≥55%

Interpretation

Decreased factor XIII (FXIII) activity could reflect acquired conditions (eg, autoimmune conditions [systemic lupus erythematosus, rheumatoid arthritis, malignancy, monoclonal gammopathy of undetermined significance, medication-isoniazid], increased consumption [surgery, disseminated intravascular coagulation/intravascular coagulation and fibrinolysis, inflammatory bowel disease, Henoch-Schonlein purpura, sepsis, thrombosis], decreased synthesis [liver disease, leukemia, medication-valproic acid and tocilizumab]), autoantibodies to FXIII, or a congenital deficiency state.

 

Normal FXIII activity makes a congenital or acquired FXIII deficiency less likely. Individuals with heterozygous FXIII-A deficiency have FXIII activity between 30% and 70% and are generally considered to be asymptomatic. However, several studies have reported severe bleeding in affected individuals.

 

Recent transfusion of plasma, cryoprecipitate, or FXIII concentrate will raise FXIII levels and may mask a diagnosis of FXIII deficiency.

Cautions

Factor XIII (FXIII) activity results may be underestimated by:

-Bilirubin greater than 7 mg/dL

-Hemoglobin greater than 125 mg/dL

-Lipemia greater than 3500 mg/dL

 

Residual platelets in frozen-thawed plasma can increase FXIII activity such that the FXIII activity may be falsely normal. Specimens that are to be frozen before testing must be centrifuged twice to remove as many of the platelets as possible before freezing.

 

Factor XIII results on Ceveron s100 are not affected by rheumatoid factor up to 600 mg/dL, fibrinogen up to 6 g/L, ammonia up to 2 mM and heparin up to 10 U/mL.

Method Description

Factor XIII (FXIII) is activated by thrombin in the presence of calcium. Activated FXIII then cleaves the sidechain carboxamide bond of the assay’s substrate and thereby releases the dark quencher (2,4-dinitrophenyl) linked to the cadaverine spacer. Subsequently, the increase of fluorescence results from the N-terminally attached fluorophore N-methyl-2-aminobenzoic acid. The fluorescent signal is proportional to the FXIII activity in the plasma sample.(Package insert: Technofluor FXIII Activity. Technoclone; 07/15/2020)

Day(s) Performed

Monday through Friday

Report Available

1 to 8 days

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.

CPT Code Information

85290

LOINC Code Information

Test ID Test Order Name Order LOINC Value
13ACT Factor XIII(13) Activity, P In Process

 

Result ID Test Result Name Result LOINC Value
623415 Factor XIII (13) Activity, P In Process