Skip to content
How It Works
Explore
Resources Ask a Question
Who It's For
Patients Caregivers Providers
Log In Ask a Question
PubMed This is a summary of 28 peer-reviewed journal articles Updated
Hematology · Acquired Factor XIII deficiency

Can You Develop Factor XIII Deficiency Later in Life?

At a Glance

Yes, adults can suddenly develop acquired Factor XIII deficiency, a rare bleeding disorder. It usually happens when the immune system mistakenly attacks the body's clotting proteins. A hallmark symptom is delayed bleeding after an injury, and it requires specialized blood tests to diagnose.

Yes, it is possible to suddenly develop Factor XIII (FXIII) deficiency later in life, though it is extremely rare [1][2]. This condition is known as acquired Factor XIII deficiency.

While congenital FXIII deficiency is a genetic disorder present at birth, the acquired form happens when something disrupts your body’s ability to maintain normal levels of Factor XIII in adulthood [3][4]. This can lead to sudden, severe bleeding symptoms in people who have never had a bleeding disorder before [5].

How the Body “Acquires” Factor XIII Deficiency

Acquired FXIII deficiency typically occurs through one of two main pathways: an autoimmune response or rapid depletion [6][7].

1. Autoimmune Destruction (Inhibitors)
In most adult cases, the condition is driven by the immune system. The immune system mistakenly identifies the body’s own Factor XIII as a threat and produces autoantibodies (proteins that attack the body’s own tissues) [8][2]. These antibodies act as “inhibitors” that neutralize or destroy Factor XIII before it can help your blood clot [1]. This autoimmune reaction is more common in older adults and can be triggered by:

  • Other Autoimmune Diseases: Conditions like Systemic Lupus Erythematosus (SLE), dermatomyositis, or rheumatoid arthritis [8][9].
  • Medications: Certain drugs, including specific cancer immunotherapies (such as pembrolizumab), have rarely been linked to triggering this response [10].
  • Underlying Malignancies: Certain blood cancers, such as leukemias, can occasionally cause the immune system to produce these inhibitors [11][12].
  • Unknown Causes (Idiopathic): In many cases, an exact trigger is never found [1].

2. Rapid Depletion (Hyperconsumption)
Sometimes, the body doesn’t attack Factor XIII, but instead uses it up faster than it can make it [13]. This is known as hyperconsumption and usually happens during extreme medical stress, such as major trauma, massive surgery, severe infections, or while on life-support machines like ECMO (extracorporeal membrane oxygenation) [5][14].

Symptoms of Acquired Factor XIII Deficiency

Because Factor XIII is responsible for stabilizing blood clots after they form, the initial clotting process often works normally, but the clot breaks down prematurely. This leads to distinct bleeding patterns:

  • Delayed Bleeding: The hallmark symptom is bleeding that starts hours or even days after a surgery, trauma, or minor injury (like a dental extraction) [15][16].
  • Spontaneous Bleeding: While delayed bleeding is most common, bleeding can also occur without any known injury or trauma.
  • Major Soft Tissue Bleeding: Large, unexplained bruising or pooling of blood in muscles (hematomas) [17][18].
  • Life-Threatening Hemorrhages: In severe cases, bleeding can occur in critical areas, such as inside the brain (intracranial hemorrhage) or the chest (hemothorax) [19][20].

The Diagnostic Challenge

If you suddenly develop severe bleeding, emergency room doctors will typically order standard blood tests, such as the PT (prothrombin time) and aPTT (activated partial thromboplastin time). In acquired FXIII deficiency, these standard tests will come back completely normal [16][5].

Because the routine tests look fine, the diagnosis is often delayed [15]. Advocacy Tip: If your routine tests are normal but you have unexplained bleeding, ask your doctor if they can check a Factor XIII activity level.

To confirm the condition, your doctor must order a specialized, quantitative Factor XIII activity assay to measure exactly how much functional FXIII is in your blood [21][22]. If levels are low, further specialized tests are run to see if autoantibodies (inhibitors) are present [23].

How is it Treated?

Treating acquired FXIII deficiency requires a two-pronged approach [24]:

  1. Stopping the Bleeding: Doctors will administer Factor XIII concentrates, cryoprecipitate (a concentrated source of clotting factors from plasma), or fresh frozen plasma to replace the missing protein and stabilize blood clots [25][26].
  2. Addressing the Underlying Cause:
    • If autoantibodies are the cause: The immune system must be suppressed so it stops destroying the new Factor XIII. This is typically done using immunosuppressive medications like corticosteroids, rituximab, or intravenous immunoglobulin (IVIG) [27][7].
    • If hyperconsumption is the cause: Doctors will focus on resolving the underlying medical stress (like an infection or trauma) to allow your body’s Factor XIII levels to recover naturally [28].

What to Expect Long-Term

A diagnosis of an acquired bleeding disorder can feel isolating and frightening. The long-term goal of immunosuppressant treatment is to achieve remission—resetting the immune system so that it completely stops producing the inhibitors [27]. Once the inhibitors are eradicated, lifelong immunosuppression is not typically required, though regular blood tests will be necessary to ensure the condition has not returned [7].

Everyday Safety: While actively managing this condition, it is critical to prioritize safety. You should wear a medical ID bracelet stating you have an acquired bleeding disorder (and noting the normal PT/aPTT labs). Always notify your dentist before any cleanings or procedures. Finally, if you experience a hard bump to the head or notice sudden, unexplained bruising, treat it as a medical emergency and head to the nearest emergency room.

Common questions in this guide

Why Does Factor XIII Only Need Monthly Infusions?Are Factor XIII Carriers at Risk During Surgery & Birth?Factor XIII Child Bumped Head? What to Do ImmediatelyWhy Do I Get Cigarette-Paper Scars with FXIII Deficiency?Does Factor XIII Deficiency Cause Permanent Joint Damage?What to Put on a Factor XIII Medical Alert BraceletHow Often Are Factor XIII Infusions in Pregnancy?Can Plasma Treat Factor XIII Deficiency Emergencies?Can I Have Factor XIII With a Normal Clot Test?Tretten vs Corifact for Factor XIII: What's the Difference?
Why are my routine bleeding tests normal if I have a bleeding disorder?
Routine tests like the PT and aPTT check the initial stages of blood clotting. Because Factor XIII works at the very end of the process to stabilize a clot after it forms, these standard tests will appear completely normal even if your Factor XIII levels are dangerously low.
What are the symptoms of acquired Factor XIII deficiency?
The hallmark symptom is delayed bleeding that starts hours or days after a surgery or minor injury. Other symptoms include sudden, unexplained bruising, large pools of blood in muscles, and severe internal bleeding without any known trauma.
How is acquired Factor XIII deficiency treated?
Treatment involves two main steps: stopping active bleeding with Factor XIII concentrates or plasma, and addressing the root cause. If the cause is an autoimmune response, immunosuppressive medications are used to stop the body from attacking its own Factor XIII.
Will I have acquired Factor XIII deficiency for the rest of my life?
Not necessarily. With proper immunosuppressive treatment, the long-term goal is to achieve remission, meaning your immune system completely stops attacking Factor XIII. Once in remission, lifelong medication is typically not required, though regular monitoring is needed.

Questions for Your Doctor

5 questions

  • •What is my current Factor XIII activity level, and do I have an active inhibitor (autoantibodies)?
  • •Could you provide a detailed emergency plan indicating exactly what symptoms should prompt an immediate trip to the ER?
  • •If I am prescribed immunosuppressants, how long do we anticipate I will need to take them, and what does the timeline to remission look like?
  • •How will we monitor my Factor XIII levels over time to ensure the autoantibodies are gone and stay gone?
  • •If I need any dental work or minor procedures in the future, what steps do we need to take beforehand to keep me safe?

Questions for You

3 questions

  • •Have I recently started any new medications, or do I have a personal history of autoimmune diseases (like lupus or rheumatoid arthritis) that I need to share with my hematologist?
  • •Are there any specific situations or activities in my daily life where I might be at risk for a bump to the head or an injury?
  • •Have I experienced any unexplained, large bruises or swelling that seemed to appear out of nowhere?

References

References (28)
  1. 1

    Severe bleeding diatheses in an elderly patient with combined type autoantibody against factor XIII A subunit; novel approach to the diagnosis and classification of anti-factor XIII antibodies.

    Kun M, Szuber N, Katona É, et al.

    Haemophilia : the official journal of the World Federation of Hemophilia 2017; (23(4)):590-597 doi:10.1111/hae.13205.

    PMID: 28345289
  2. 2

    Acquired Factor XIII inhibitor associated with mantle cell lymphoma.

    Nixon CP, Prsic EH, Guertin CA, et al.

    Transfusion 2017; (57(3)):694-699 doi:10.1111/trf.13947.

    PMID: 27917497
  3. 3

    Comparison of F13A1 gene mutations in 73 patients treated with recombinant FXIII-A2.

    Ivaškevičius V, Biswas A, Garly ML, Oldenburg J

    Haemophilia : the official journal of the World Federation of Hemophilia 2017; (23(3)):e194-e203 doi:10.1111/hae.13233.

    PMID: 28520207
  4. 4

    Gynecological and obstetric outcome in the French cohort of women with factor XIII deficiency.

    Rugeri L, Martinaud C, Beurrier P, et al.

    Thrombosis research 2020; (191()):22-25 doi:10.1016/j.thromres.2020.04.010.

    PMID: 32360976
  5. 5

    Acquired factor XIII deficiency in two patients with bleeding events during veno-venous extracorporeal membrane oxygenation treatment.

    Ito A, Iwashita Y, Esumi R, et al.

    Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs 2020; (23(3)):283-287 doi:10.1007/s10047-019-01148-w.

    PMID: 31834529
  6. 6

    Frequent bleeding symptoms associated with autoimmune acquired factor XIII/13 deficiency due to anti-factor XIII A and B subunit antibodies.

    Ozaki S, Mizuguchi M, Okamoto Y, et al.

    American journal of hematology 2022; (97(11)):1497-1500 doi:10.1002/ajh.26685.

    PMID: 35957554
  7. 7

    Acquired factor XIII deficiency: A review.

    Yan MTS, Rydz N, Goodyear D, Sholzberg M

    Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2018; (57(6)):724-730 doi:10.1016/j.transci.2018.10.013.

    PMID: 30446212
  8. 8

    A Case of Acquired Factor XIII Deficiency with Systemic Lupus Erythematosus Diagnosed after Repeated Intracerebral Hemorrhages.

    Okumura E, Onuki H, Otsuka K, et al.

    NMC case report journal 2023; (10()):121-124 doi:10.2176/jns-nmc.2022-0373.

    PMID: 37293197
  9. 9

    A case of anti-NPX-2 antibody-positive dermatomyositis resulting in massive haemothorax with acquired factor XIII deficiency.

    Matsuda T, Haga T, Sakaguchi T, et al.

    Modern rheumatology case reports 2023; (8(1)):91-94 doi:10.1093/mrcr/rxad049.

    PMID: 37606624
  10. 10

    A Rare Case of Factor XIII Deficiency in the Setting of Cancer Immunotherapy.

    Farzam K

    Cureus 2021; (13(5)):e15299 doi:10.7759/cureus.15299.

    PMID: 34084689
  11. 11

    Acquired factor XIII deficiency in myeloid neoplasms: case series and review of literature.

    Haroon A, Mohammed Saleh MF, Alahmari A, et al.

    Hematology/oncology and stem cell therapy 2024; (17(4)):245-247 doi:10.4103/hemoncstem.hemoncstem-D-24-00034.

    PMID: 39829100
  12. 12

    Acquired factor XIII deficiency in a child with pure erythroid leukemia.

    Feugray G, Buchbinder N, Buchonnet G, et al.

    Pediatric blood & cancer 2021; (68(6)):e28890 doi:10.1002/pbc.28890.

    PMID: 33484080
  13. 13

    Acquired factor XIII deficiency in adult patients during ECMO: a prospective observational study.

    Noitz M, Jenner D, Tschoellitsch T, et al.

    Scientific reports 2025; (15(1)):39110 doi:10.1038/s41598-025-26452-9.

    PMID: 41203730
  14. 14

    Acquired Factor XIII Deficiency in Patients with Multiple Trauma.

    Hetz M, Juratli T, Tiebel O, et al.

    Injury 2023; (54(5)):1257-1264 doi:10.1016/j.injury.2022.12.021.

    PMID: 36577625
  15. 15

    Nonimmune-acquired factor XIII deficiency: a cause of high volume and delayed postoperative hemorrhage.

    Chuliber FA, Schutz NP, Viñuales ES, et al.

    Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 2020; (31(8)):511-516 doi:10.1097/MBC.0000000000000953.

    PMID: 32852328
  16. 16

    [A Case of Autoimmune Acquired Factor XIII Deficiency Diagnosed from Recurrent Postoperative Bleeding].

    Takashima Y, Kawanishi H, Kotani S, et al.

    Hinyokika kiyo. Acta urologica Japonica 2023; (69(6)):169-173 doi:10.14989/ActaUrolJap_69_6_169.

    PMID: 37460281
  17. 17

    Acquired Factor XIII Deficiency Inducing Recurrent and Fatal Bleeding, Description of a Case.

    Di Micco P, Gussoni G, Pieralli F, et al.

    Journal of blood medicine 2020; (11()):43-45 doi:10.2147/JBM.S232115.

    PMID: 32099500
  18. 18

    Auto- and alloantibodies against factor XIII: laboratory diagnosis and clinical consequences.

    Muszbek L, Pénzes K, Katona É

    Journal of thrombosis and haemostasis : JTH 2018; (16(5)):822-832 doi:10.1111/jth.13982.

    PMID: 29460500
  19. 19

    Factor XIII deficiency with intracranial haemorrhage.

    Ejaz M, Saleem A, Ali N, Tariq F

    BMJ case reports 2019; (12(8)) doi:10.1136/bcr-2018-228682.

    PMID: 31451452
  20. 20

    Recurrent cortical intracranial hemorrhage secondary to factor XIII deficiency: case report and literature review.

    Farasati M, Hosseini SA, Baghestani Z

    Brain injury 2026; (40(4)):296-302 doi:10.1080/02699052.2025.2612601.

    PMID: 41482940
  21. 21

    Measurement of factor XIII for the diagnosis and management of deficiencies: insights from a retrospective review of 10 years of data on consecutive samples and patients.

    Al Sharif MA, Mathews N, Tasneem S, et al.

    Research and practice in thrombosis and haemostasis 2025; (9(1)):102689 doi:10.1016/j.rpth.2025.102689.

    PMID: 40027442
  22. 22

    State of the art in factor XIII laboratory assessment.

    Durda MA, Wolberg AS, Kerlin BA

    Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2018; (57(6)):700-704 doi:10.1016/j.transci.2018.07.006.

    PMID: 30087086
  23. 23

    Agreement between factor XIII activity and antigen assays in measurement of factor XIII: A French multicenter study of 147 human plasma samples.

    Caron C, Meley R, Le Cam Duchez V, et al.

    International journal of laboratory hematology 2017; (39(3)):279-285 doi:10.1111/ijlh.12621.

    PMID: 28318109
  24. 24

    Massive recurrent post-tonsillectomy bleedings revealing a transient factor XIII deficiency in a 10-year-old boy. A case report.

    Jankovic M, Choucair ML, Hallak B, et al.

    International journal of pediatrics & adolescent medicine 2019; (6(2)):55-57 doi:10.1016/j.ijpam.2019.05.006.

    PMID: 31388547
  25. 25

    Congenital Factor XIII Deficiency With the Presence of Inhibitor: A Case Study.

    Karaman S, Akkaya E, Genc S, et al.

    Journal of pediatric hematology/oncology 2021; (43(1)):e99-e102 doi:10.1097/MPH.0000000000001671.

    PMID: 31764516
  26. 26

    The impact of acquired coagulation factor XIII deficiency in traumatic bleeding and wound healing.

    Kleber C, Sablotzki A, Casu S, et al.

    Critical care (London, England) 2022; (26(1)):69 doi:10.1186/s13054-022-03940-2.

    PMID: 35331308
  27. 27

    [Acquired autoimmune coagulation factor XIII/13 deficiency].

    Ogawa Y

    [Rinsho ketsueki] The Japanese journal of clinical hematology 2020; (61(7)):799-808 doi:10.11406/rinketsu.61.799.

    PMID: 32759568
  28. 28

    Factor XIII Deficiency: A Review of Clinical Presentation and Management.

    Pelcovits A, Schiffman F, Niroula R

    Hematology/oncology clinics of North America 2021; (35(6)):1171-1180 doi:10.1016/j.hoc.2021.07.009.

    PMID: 34607717

This page provides educational information about acquired Factor XIII deficiency. Always consult a hematologist or seek emergency medical care for sudden or unexplained bleeding.

Get notified when new evidence is published on Factor XIII Deficiency.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.