Hageman’s disease (hereditary factor XII deficiency). Review of literature, clinical observations
https://doi.org/10.21682/2311-1267-2024-11-3-66-70
Abstract
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.
Supporting agencies: The study was performed without external funding
About the Authors
A. A. Polikarpova
Kirov Research Institute of Hematology and Blood Transfusion under the Federal Medical Biological Agency
Russian Federation
Russian Federation
Pediatric Hematologist.
72 Krasnoarmeyskaya St., Kirov, 610027
S. V. Ignatiev
Kirov Research Institute of Hematology and Blood Transfusion under the Federal Medical Biological Agency
Russian Federation
Russian Federation
Cand. Of Sci. (Med.), Senior Resercher of Department of Organization and Support of Scientific Research.
72 Krasnoarmeyskaya St., Kirov, 610027
A. V. Lyanguzov
Kirov Research Institute of Hematology and Blood Transfusion under the Federal Medical Biological Agency
Russian Federation
Russian Federation
Cand. Of Sci. (Med.), Senior Resercher of Department of Organization and Support of Scientific Research.
72 Krasnoarmeyskaya St., Kirov, 610027
P. A. Zharkov
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation
Russian Federation
Dr. of Sci. (Med.), Pediatrician, Hematologist Outpatient Consultative Unit, Head of the Hemostasis Pathology Department, Web of Science ResearcherID: AAP-9203-2020.
1 Samory Mashela St., Moscow, 117997
O. M. Tselousova
Kirov Research Institute of Hematology and Blood Transfusion under the Federal Medical Biological Agency
Russian Federation
Russian Federation
Cand. Of Sci. (Med.), Head of the Children’s Hematology and Chemotherapy Department.
72 Krasnoarmeyskaya St., Kirov, 610027
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Review
For citations:
Polikarpova A.A., Ignatiev S.V., Lyanguzov A.V., Zharkov P.A., Tselousova O.M. Hageman’s disease (hereditary factor XII deficiency). Review of literature, clinical observations. Russian Journal of Pediatric Hematology and Oncology. 2024;11(3):66-70. (In Russ.) https://doi.org/10.21682/2311-1267-2024-11-3-66-70
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