Test Details
Methodology
FXIII contained in the sample is converted by the action of thrombin into FXIIIa. Fibrin formed by thrombin also accelerates this reaction. Fibrinogen is not removed prior to testing since this would cause a loss of FXIII. Instead, fibrin produced by the action of thrombin is prevented from forming clots by an aggregation inhibiting peptide and held in solution. FXIIIa cross-links a specific peptide substrate to glycine ethyl ester, thereby releasing ammonia. The ammonia released is then determined in a parallel enzymatic reaction. The decrease in NADH is measured by monitoring the absorbance at 340 nm.
Result Turnaround Time
4 - 6 days
Turnaround time is defined as the usual number of days from the date of pickup of a specimen for testing to when the result is released to the ordering provider. In some cases, additional time should be allowed for additional confirmatory or additional reflex tests. Testing schedules may vary.
Use
This test is used to measure the level of factor XIII activity in citrate plasma.
Special Instructions
If the patient's hematocrit exceeds 55%, the volume of citrate in the collection tube must be adjusted. Refer to Coagulation Collection Procedures for directions.
Limitations
This test was developed and its performance characteristics determined by Labcorp. It has not been cleared or approved by the Food and Drug Administration.
Custom Additional Information
Factor XIII (FXIII) is a protein involved in blood clot stabilization, which also plays an important role in processes including trauma, wound healing, tissue repair, pregnancy and even bone metabolism.1-6 In cases of FXIII deficiency, routine coagulation tests such as prothrombin time, activated partial thromboplastin time and thrombin time remain within their reference ranges, so it is essential to apply specific tests to assess FXIII levels.1-4,7
Factor XIII is found in plasma and platelets.2,9 Plasma factor XIII consists of 2 A-subunits and 2 B-subunits, while platelet factor XIII consists of only 2 A-subunits.2,3,8,9 After factor XIII 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 cross-linked exhibits an increased susceptibility to fibrinolysis. FXIII also exerts antifibrinolytic activity through cross-linking of α2-antiplasmin to fibrin.10-13
Though extremely rare in occurrence, congenital disorders of Factor XIII are associated with high rates of morbidity and mortality.2,3,8 Congenital factor XIII deficiency is an autosomal recessive bleeding disorder. Homozygous individuals (FXIII <1%) experience soft tissue hemorrhage, hemarthrosis and hematomas.1-3 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 is also observed. Heterozygous carriers may be asymptomatic; however, females may experience recurrent spontaneous abortions.
Acquired factor XIII (FXIII) deficiency is a rare bleeding disorder that can manifest with spontaneous or delayed life-threatening hemorrhage. Causes of acquired deficiency include immune-mediated inhibition, as well as non-immune FXIII hyper-consumption or diminished synthesis.14-19 The occurrence of acquired FXIII deficiency can be idiopathic or may be associated with comorbidities, such as malignancies or autoimmune disorders.17
Specimen Requirements
Specimen
Plasma, frozen
Volume
1 mL
Minimum Volume
0.5 mL (Note: This volume does not allow for repeat testing.)
Container
Blue-top (sodium citrate) tubes
Collection Instructions
Blood should be collected in a blue-top tube containing 3.2% buffered sodium citrate.20 Evacuated collection tubes must be filled to completion to ensure a proper blood-to-anticoagulant ratio.21,22 The sample should be mixed immediately by gentle inversion at least six times to ensure adequate mixing of the anticoagulant with the blood. A discard tube is not required prior to collection of coagulation samples unless the sample is collected using a winged (butterfly) collection system. With a winged blood collection set, a discard tube should be drawn first to account for the dead space of the tubing and prevent under-filling of the evacuated tube.23,24 When noncitrate tubes are collected for other tests, collect sterile and nonadditive (red-top) tubes prior to citrate (blue-top) tubes. Any tube containing an alternative anticoagulant should be collected after the blue-top tube. Gel-barrier tubes and serum tubes with clot initiators should also be collected after the citrate tubes.
Please print and use the Volume Guide for Coagulation Testing to ensure proper draw volume.
Reference Range
43–148%
Storage Instructions
Freeze.
References
Bouttefroy S, Meunier S, Milien V, et al. Congenital factor XIII deficiency: comprehensive overview of the FranceCoag cohort. Br J Haematol. 2020 Jan;188(2):317-320. PubMed 31414482
Byrnes JR, Wolberg AS. Newly-Recognized Roles of Factor XIII in Thrombosis. Semin Thromb Hemost. 2016 Jun;42(4):445-454. PubMed 27056150
Dull K, Fazekas F, Törőcsik D. Factor XIII-A in Diseases: Role Beyond Blood Coagulation. Int J Mol Sci. 2021 Feb 1;22(3):1459. PubMed 33535700
Farzam K. A Rare Case of Factor XIII Deficiency in the Setting of Cancer Immunotherapy. Cureus. 2021 May 28;13(5):e15299. PubMed 34084689
Jain S, Acharya SS. Management of rare coagulation disorders in 2018. Transfus Apher Sci. 2018 Dec;57(6):705-712. PubMed 30392819
Komáromi I, Bagoly Z, Muszbek L. Factor XIII: novel structural and functional aspects. J Thromb Haemost. 2011 Jan;9(1):9-20. PubMed 20880254
Menegatti M, Peyvandi F. Treatment of rare factor deficiencies other than hemophilia. Blood. 2019 Jan 31;133(5):415-424. PubMed 30559262
Muszbek L, Katona É, Kerényi A. Assessment of Factor XIII. Methods Mol Biol. 2017;1646:277-293. PubMed 28804836
Footnotes
1. Malkhassian D, Sabir S, Sharma S. Physiology, Factor XIII. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan. 2023 Nov 5. PubMed 30855859
2. Durda MA, Wolberg AS, Kerlin BA. State of the art in factor XIII laboratory assessment. Transfus Apher Sci. 2018 Dec;57(6):700-704. PubMed 30087086
3. Tahlan A, Ahluwalia J. Factor XIII: congenital deficiency factor XIII, acquired deficiency, factor XIII A-subunit, and factor XIII B-subunit. Arch Pathol Lab Med. 2014 Feb;138(2):278-281. PubMed 24476525
4. Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L, Factor XIII and Fibrinogen SSC Subcommittee of the ISTH. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011 Jul;9(7):1404-1406. PubMed 22946956
5. Muszbek L, Bereczky Z, Bagoly Z, Komáromi I, Katona E. Factor XIII: a coagulation factor with multiple plasmatic and cellular functions. Physiol Rev. 2011 Jul;91(3):931-972. PubMed 21742792
6. Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia. 2008 Nov;14(6):1190-1200. PubMed 19141159
7. Karimi M, Peyvandi F, Naderi M, Shapiro A. Factor XIII deficiency diagnosis: Challenges and tools. Int J Lab Hematol. 2018 Feb;40(1):3-11. PubMed 29027765
8. Mahečić TT, Konosić S, Noitz M, Bobinac M. Coagulation Factor XIII - Last to think about? Blood Transfus. 2025 Jan;23(1):70-74. PubMed 39804745
9. Mitchell JL, Mutch NJ. Let's cross-link: diverse functions of the promiscuous cellular transglutaminase factor XIII-A. J Thromb Haemost. 2019 Jan;17(1):19-30. PubMed 30489000
10. Mitchell JL, Wright S, Kazi S, Watson HG, Mutch NJ. Defective α2 antiplasmin cross-linking and thrombus stability in a case of acquired factor XIII deficiency. Br J Haematol. 2017 Sep;178(5):794-799. PubMed 28516512
11. Rijken DC, Abdul S, Malfliet JJMC, Leebeek FWG, Uitte de Williage S. Compaction of fibrin clots reveals the antifibrinolytic effect of factor XIII. J Thromb Haemost. 2016 Jul;14(7):1453-1461. PubMed 27148673
12. Schroeder V, Kohler HP. New developments in the area of factor XIII. J Thromb Haemost. 2013 Feb;11(2):234-244. PubMed 23279671
13. Fraser SR, Booth NA, Mutch NJ. The antifibrinolytic function of factor XIII is exclusively expressed through α2-antiplasmin cross-linking. Blood. 2011 Jun 9;117(23):6371-6374. PubMed 21471521
14. Duranteau O, Tatar G, Demulder A, Tuna T. Acquired factor XIII deficiency: A scoping review. Eur J Anaesthesiol Intensive Care. 2023 Sep 29;2(5):e0035. PubMed 39916809
15. Kleber C, Sablotzki A, Casu S, et al. The impact of acquired coagulation factor XIII deficiency in traumatic bleeding and wound healing. Crit Care. 2022 Mar 24;26(1):69. PubMed 35331308
16. Ichinose A, Japanese Collaborative Research Group on AH13. Autoimmune acquired factor XIII deficiency dueto anti-factor XIII/13 antibodies: A summary of 93 patients. Blood Rev. 2017 Jan;31(1):37-45. PubMed 27542511
17. Yan MTS, Rydz N, Goodyear D, Sholzberg M. Acquired factor XIII deficiency: A review. Transfus Apher Sci. 2018 Dec;57(6):724-730. PubMed 30446212
18. Franchini M, Frattini F, Crestani S, Bonfanti C. Acquired FXIII inhibitors: a systematic review. J Thromb Thrombolysis. 2013 Jul;36(1):109-114. PubMed 23065324
19. Marco A, Marco P. Autoimmune Acquired Factor XIII Deficiency: A Case Report. J Blood Med. 2021 Feb 9;12:63-68. PubMed 33603527
20. Adcock DM, Kressin DC, Marlar RA. Effect of 3.2% vs 3.8% sodium citrate concentration on routine coagulation testing. Am J Clin Pathol. 1997 Jan;107(1):105-110. PubMed 8980376
21. Reneke J, Etzell J, Leslie S, Ng VL, Gottfried EL. Prolonged prothrombin time and activated partial thromboplastin time due to underfilled specimen tubes with 109 mmol/L (3.2%) citrate anticoagulant. Am J Clin Pathol. 1998 Jun;109(6):754-757. PubMed 9620035
22. National Committee for Clinical Laboratory Standardization. Collection, Transport, and Processing of Blood Specimens for Coagulation Testing and General Performance of Coagulation Assays; Approved Guideline. 5th ed. Villanova, Pa: NCCLS; 2008. Document H21-A5:28(5).
23. Gottfried EL, Adachi MM. Prothrombin time ad activated partial thromboplastin time can be performed on the first tube. Am J Clin Pathol. 1997 Jun;107(6):681-683. PubMed 9169665
24. McGlasson DL, More L, Best HA, Norris WL, Doe RH, Ray H. Drawing specimens for coagulation testing: Is a second tube necessary? Clin Lab Sci. 1999 May-Jun;12(3):137-139. PubMed 10539100
LOINC® Map
| Order Code | Order Code Name | Order Loinc | Result Code | Result Code Name | UofM | Result LOINC |
|---|---|---|---|---|---|---|
| 086340 | Factor XIII Activity | 27815-0 | 086341 | Factor XIII Activity | % | 27815-0 |
| Order Code | 086340 | |||||
| Order Code Name | Factor XIII Activity | |||||
| Order Loinc | 27815-0 | |||||
| Result Code | 086341 | |||||
| Result Code Name | Factor XIII Activity | |||||
| UofM | % | |||||
| Result LOINC | 27815-0 |