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Russian Journal of Pediatric Hematology and Oncology

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Factor XIII deficiency – where we can save

https://doi.org/10.21682/2311-1267-2021-8-4-90-94

Abstract

Factor XIII deficiency is one of the rare bleeding disorders with an approximate prevalence about 1 per 3 million. A characteristic feature of this deficiency is the high frequency of severe bleeding, including intracranial hemorrhages. This coagulopathy is not detected using a standard coagulation tests, measuring of factor XIII activity is required. The world practice of treating this hemorrhagic condition is the use of plasma and recombinant concentrate. These drugs are not registered in the territory of the Russian Federation. In our country, cryoprecipitate is used. This article describes the clinical case, diagnosis and unsatisfactory results of cryoprecipitate therapy in a patient with diagnosis of factor XIII deficiency. Prompt registration of the XIII factor concentrates is required.

About the Authors

D. B. Florinsky
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation

D.B. Florinsky: Resident in Pediatrics

117198, Moscow, Samory Mashela St., 1



A. V. Pshonkin
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation

A.V. Pshonkin: Cand. of Sci. (Med.), Head of the Hospital for Short-Term Treatment
SPIN-code: 6270-4864

117198, Moscow, Samory Mashela St., 1



A. V. Poletaev
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation

A.V. Poletaev: Head of the Laboratory of Clinical Hemostasis
Web of Science ResearcherID: AAW-9954-2020

117198, Moscow, Samory Mashela St., 1



D. V. Fedorova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation

D.V. Fedorova: Cand. of Sci. (Med.), Hematologist of Outpatient Consultative Unit, Research Associate of the Department of Hemostasis Research

117198, Moscow, Samory Mashela St., 1



E. A. Seregina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia; Center for Theoretical Problems of Physicochemical Pharmacology of the Russian Academy of Sciences
Russian Federation

E.A. Seregina: Cand. of Sci. (Biol.), Research Associate Laboratory of Clinical Hemostasis of Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology; Research Assistant Center for Theoretical Problems of Physicochemical Pharmacology of the Russian Academy of Sciences
Web of Science ResearcherID: A-7499-2014

117198, Moscow, Samory Mashela St., 1
109029, Moscow, Srednyaya Kalitnikovskaya St., 30



E. V. Ursulenko
Irkutsk State Regional Children’s Clinical Hospital
Russian Federation

E.V. Ursulenko: Hematologist of Consulting and Diagnostic Center

664022, Irkutsk, Gagarin Blvd., 4 



O. P. Tolmacheva
Irkutsk State Regional Children’s Clinical Hospital
Russian Federation

O.P. Tolmacheva: Hematologist of the Oncology Department

664022, Irkutsk, Gagarin Blvd., 4 



P. A. Zharkov
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia
Russian Federation

P.A. Zharkov: Dr. of Sci. (Med.), Pediatrician, Hematologist Outpatient Consultative Unit, Head of the Department of Hemostasis Research
Web of Science ResearcherID: AAP-9203-2020

117198, Moscow, Samory Mashela St., 1



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For citations:


Florinsky D.B., Pshonkin A.V., Poletaev A.V., Fedorova D.V., Seregina E.A., Ursulenko E.V., Tolmacheva O.P., Zharkov P.A. Factor XIII deficiency – where we can save. Russian Journal of Pediatric Hematology and Oncology. 2021;8(4):90-94. (In Russ.) https://doi.org/10.21682/2311-1267-2021-8-4-90-94

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ISSN 2311-1267 (Print)
ISSN 2413-5496 (Online)
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