Hageman’s disease (hereditary factor XII deficiency). Review of literature, clinical observations
Hageman’s disease is a rare hereditary disorder of hemostasis, characterized by a deficiency of coagulation factor XII, accompanied by prolongation of blood clotting time (Lee–White) and activated partial thromboplastin time. These laboratory abnormalities are often thought to indicate an increased risk of bleeding, similar to hemophilia. This leads to the unnecessary use of transfusions and hemostatic therapy, since bleeding is rare in these patients. The article provides a review of the literature on Hageman’s disease and describes clinical cases of surgical treatment of children with factor XII deficiency.
Authors
- S. V. Ignatyev (ORCID: https://orcid.org/0000-0002-8457-2967)
- A. V. Lyanguzov (ORCID: https://orcid.org/0000-0001-5509-5308)
- P. А. Zharkov (ORCID: https://orcid.org/0000-0003-4384-6754)
- О. М. Целоусова (ORCID: https://orcid.org/0000-0001-6944-0344)
- A. A. Polikarpova
Institutions
- Kirov Research Institute of Hematology and Blood Transfusion FMBA (RU)
- Ministry of Health of the Russian Federation (RU)
- Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology (RU)
- Federal Medical-Biological Agency (RU)
Publication Details
- Journal
- Russian Journal of Pediatric Hematology and Oncology
- Published
- 2024-10-30
- DOI
- https://doi.org/10.21682/2311-1267-2024-11-3-66-70
- Primary Topic
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Type
- article
- Field-Weighted Citation Impact
- 0.00