ArticleInternational journal of hematology2015
Report of a patient with chronic intractable autoimmune hemorrhaphilia due to anti-factor XIII/13 antibodies who died of hemorrhage after sustained clinical remission for 3 years.
Article in International journal of hematology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 14 citations in OpenAlex.
- Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan.International journal of hematology · 2024Article
- Acquired factor XIII deficiency: A scoping review.European journal of anaesthesiology and intensive care · 2023Review
- Measuring Factor XIII Inhibitors in Patients with Factor XIII Deficiency: A Case Report and Systematic Review of Current Practices in Japan.Journal of clinical medicine · 2022Review
- A case of life-threatening small intestinal bleeding accompanied by lower coagulation factor XIII activity.Clinical journal of gastroenterology · 2020Article
- Successful bypass surgery for esophageal carcinoma under adequate factor XIII/13 replacement therapy in a case of intractable autoimmune hemorrhaphilia due to anti-Factor XIII/13 antibodies.International journal of hematology · 2016Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
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Abstract
Although the incidence of autoimmune hemorrhaphilia due to anti-Factor XIII (FXIII, not FVIII or FXII to avoid confusion) antibodies (AH13) or hemorrhagic "acquired FXIII deficiency due to anti-FXIII autoantibodies" was previously considered rare, it has been on the increase in the twenty-first century, at least in Japan. An 83-year-old woman with an unexplained hemorrhage was admitted to our hospital for intramuscular hematoma and severe anemia. Her FXIII activity was reduced to 10 % of normal; since FXIII inhibitors and anti-FXIII-A subunit autoantibodies were detected, she was definitively diagnosed with AH13. Despite developing cardiac tamponade due to pericardial hemorrhage, she clinically recovered from AH13 after hemostatic therapy with FXIII-concentrates and immunosuppressive treatment with rituximab and cyclophosphamide. However, her FXIII activity remained low and she died of hemorrhage 3.5 years after admission. AH13 patients should be monitored for a prolonged period, as this disease is very likely a chronic intractable hemorrhagic disorder.
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