Evidence map›Paper›PMID 41583548›Full record

ReviewClinical hematology international2026

Factor XIII Deficiency: A Review of Biology, Testing, and Treatment.

Jeremy W Jacobs, Garrett S Booth, Victoria Costa, Cristina A Figueroa Villalba, Bipin N Savani, Brian D Adkins

Abstract readReview
In one paragraph

Review in Clinical hematology international, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed.

  1. Article
  2. Review
  3. Review
  4. Article
  5. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Jeremy W JacobsDepartment of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN, USA.ORCID https://orcid.org/0000-0002-5719-9685
Garrett S BoothDepartment of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN, USA.ORCID https://orcid.org/0000-0002-8422-9480
Victoria CostaDepartment of Pathology, NYU Grossman School of Medicine, New York, NY, USA.
Cristina A Figueroa VillalbaDepartment of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA.
Bipin N SavaniDivision of Hematology-Oncology, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.ORCID https://orcid.org/0000-0002-3304-9965
Brian D AdkinsDivision of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA.ORCID https://orcid.org/0000-0002-9480-2762

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Factor XIII (FXIII) deficiency is a rare bleeding disorder characterized by unstable hemostatic clots due to defective fibrin cross‑linking. Congenital FXIII deficiency arises from variants in the F13A1 (FXIII-A subunit) or F13B (FXIII-B subunit) genes, and classically presents with delayed umbilical stump hemorrhage, soft‑tissue and intracranial bleeding, impaired wound healing, and recurrent pregnancy loss. Acquired deficiency stems from inhibitory autoantibodies or from reduced synthesis or consumption in critical illness and surgery. Routine coagulation screening tests are normal and diagnosis relies on quantitative FXIII activity assays with or without antigenic phenotyping and, when indicated, inhibitor testing and molecular confirmation. Plasma‑derived FXIII concentrate reduces spontaneous and intracranial bleeding; recombinant FXIII‑A2 is appropriate for F13A1 defects but not patients with F13B variants. Perioperative and obstetric care target activity thresholds suited to procedural risk and individual pharmacokinetics. This review synthesizes the molecular biology, epidemiology, clinical features, diagnostic methods, and evidence‑based management of FXIII deficiency, with practical guidance for assay selection, validation, and result interpretation.

Indexed as

Bleeding disorderCryoprecipitateDelayed bleedingFactor deficiencyFactor XIIIFactor XIII deficiencyFXIIIHemostasisLaboratory testing

Identifiers

PMID41583548
PMCPMC12825037

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.