Железодефицит и болезнь Виллебранда: взаимосвязь и междисциплинарный подход

Виноградова М.А. Железодефицит и болезнь Виллебранда: взаимосвязь и междисциплинарный подход. Гинекология. 2025;27(3):183–189. DOI: 10.26442/20795696.2025.3.203410

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Vinogradova MA. Iron deficiency and von Willebrand disease: the relationship and interdisciplinary approach. A review and own data. Gynecology. 2025;27(3):183–189. DOI: 10.26442/20795696.2025.3.203410

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Аннотация

Железодефицитные состояния широко распространены в клинической практике и особенно часто возникают у женщин репродуктивного возраста. При коагулопатиях хроническая кровопотеря, в частности обильные менструальные кровотечения, становится основной причиной развития железодефицитной анемии. Самая распространенная форма коагулопатии – болезнь Виллебранда, и если тяжелые ее формы обычно устанавливаются с детского возраста, то нетяжелые могут длительно протекать скрыто. Железодефицит не только ухудшает качество жизни больных, но и может осложнять течение основного заболевания, создавая порочный круг. Ведение таких больных представляет собой клинический вызов и требует комплексного междисциплинарного подхода: своевременной диагностики коагулопатии, оценки объема кровопотери, его коррекции, подбора оптимальной схемы восполнения дефицита железа и обязательного устранения гемостазиологического нарушения. В статье рассматриваются современные принципы диагностики и терапии железодефицита у женщин с коагулопатиями, представлена модель пациента для превентивного подхода. Основа терапии при неустраненной избыточной кровопотере – препараты железа для парентерального введения, в частности карбоксимальтозат железа. Именно своевременное назначение адекватных доз хорошо переносимого препарата железа, ставшее возможным с появлением карбоксимальтозата железа, позволяет не допустить тяжелых форм анемии и улучшить качество жизни пациенток.

Ключевые слова: железодефицит, анемия, коагулопатия, обильное менструальное кровотечение, болезнь Виллебранда, ферритин, карбоксимальтозат железа, переносимость препарата железа

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Iron deficiency conditions are common for clinical practice especially in women of reproductive age. In coagulopathies, chronic blood loss, in particular heavy menstrual bleeding, becomes the main cause of iron deficiency anemia. The most common form of coagulopathy is von Willebrand disease, and if its severe forms are established in childhood, then mild ones can proceed latent for a long time. Iron deficiency not only worsens the quality of life but can also complicate the course of the underlying disease, creating a vicious circle. Management of such patients is a clinical challenge and requires a comprehensive interdisciplinary approach: timely diagnosis, assessment of the volume of blood loss, its correction, selection of the optimal scheme for replenishing iron deficiency and mandatory elimination of the main hemostasis disorder. This article discusses modern principles of diagnosis and therapy of iron deficiency in women with coagulopathies. The patient model for a preventive approach is presented. The basis of therapy in case of persistent excessive blood loss are iron preparations for parenteral administration, in particular, ferric carboxymaltose. The timely administration of adequate doses of a well-tolerated iron preparation, which became possible with the advent of ferric carboxymaltose, makes it possible to prevent severe forms of anemia and improve the patients’ quality of life.

Keywords: iron deficiency, anemia, coagulopathy, heavy menstrual bleeding, von Willebrand disease, ferritin, ferric carboxymaltose, tolerability of iron preparation

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Список литературы

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23. VanderMeulen H, Tang GH, Sholzberg M. Tranexamic acid for management of heavy vaginal bleeding: barriers to access and myths surrounding its use. Res Pract Thromb Haemost. 2024;8(3):e102389 DOI:10.1016/j.rpth.2024.102389
24. Committee on Adolescent Health Care; Committee on Gynecologic Practice. Committee Opinion No. 580: Von Willebrand disease in women. Obstet Gynecol. 2013;122:1368-73. DOI:10.1097/01.AOG.0000438961.38979.19
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1. Dobrokhotova YuE, Kuznetsova OV. Iron deficiency anemia in obstetric and gynecological practice. Akusherstvo i Ginekologiia. 2016;8:10-5 (in Russian). DOI:10.18565/aig.2016.8.10-15
2. Myrin-Westesson L, Elfvinge P, Zetterberg E, Olsson A. Prevalence of heavy menstrual bleeding, iron deficiency, iron deficiency anemia, and treatment in women with von Willebrand disease: a cohort study. Res Pract Thromb Haemost. 2025;9(4):102949. DOI:10.1016/j.rpth.2025.102949
3. Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease – 2023 executive summary. Blood Adv. 2021;5(1):301-25. DOI:10.1182/bloodadvances.2020003264
4. American College of Obstetricians and Gynecologists. Screening and Management of Bleeding Disorders in Adolescents With Heavy Menstrual Bleeding. Committee Opinion No. 785. Obstet Gynecol. 2019;134(3):e71-83. DOI:10.1097/AOG.0000000000003411
5. Lavin M, Aguila S, Dalton N, et al. Significant gynecological bleeding in women with low von Willebrand factor levels. Blood Advances. 2018;2(14):1784-91. DOI:10.1182/bloodadvances.2018017418
6. MacLean B, Sholzberg M, Weyand AC, et al. Identification of women and girls with iron deficiency in the reproductive years. Int J Gynecol Obstet. 2023;162(Suppl. 2):58-67. DOI:10.1002/ijgo.14948
7. Snook J, Bhala N, Beales I, et al. BSG guideline for management of iron deficiency anemia in adults. Gut. 2021;70(11):2030-51. DOI:10.1136/gutjnl-2021-325210
8. Zyryanov SK, Baybulatova EA. Selection of parenteral iron supplement for iron deficiency anemia: A review. Terapevticheskii Arkhiv (Ter. Arkh.). 2024;96(4):407-18 (in Russian). DOI:10.26442/00403660.2024.04.202693
9. Turan O, Gomez K, Kadir RA. Review of interventions and effectiveness for heavy menstrual bleeding in women with moderate and severe von Willebrand disease. Haemophilia. 2024;30:1177-84. DOI:10.1111/hae.15078
10. Zozulya NI, Yastrubinetskaya OI. Compliance with therapy in patients with von Willebrand's disease. Voprosy Gematologii/Onkologii i Immunopatologii v Pediatrii. 2022;21(1):83-7 (in Russian). DOI:10.24287/1726-1708-2022-21-1-83-87
11. Munro MG, Mast AE, Powers JM, et al. The relationship between heavy menstrual bleeding, iron deficiency and iron-deficiency anemia. Am J Obstet Gynecol. 2023;229(1):1-9. DOI:10.1016/j.ajog.2023.01.017
12. Zoller H, Schaefer B, Blumenstein I, et al. Hypophosphataemia after ferric carboxymaltose vs ferric derisomaltose (PHOSPHARE-IBD): randomized trial. Gut. 2023;72(4):644-53. DOI:10.1136/gutjnl-2022-327897
13. Injectafer® (Ferric Carboxymaltose). Prescribing Information. U.S. FDA label (2021–2024 updates).
14. Ferinject® (Ferric Carboxymaltose). Product Monograph. CSL Behring; 2024.
15. Vinogradova MA. Ferric carboxymaltose in gynaecological practice. Clin Pharmacol Ther. 2014;23(4):1-5 (in Russian).
16. Akpan IJ, Narang M, Zampaglione E, et al. Iron deficiency anemia in patients with heavy menstrual bleeding: The patients’ perspective from diagnosis to treatment. Women’s Health (Lond). 2025;21:17455057251321221. DOI:10.1177/17455057251321221
17. Secilmis S, Iskender D, Candir BA, et al. Efficiency of intravenous iron carboxymaltose in patients with iron-deficiency anemia due to heavy menstrual bleeding: a single-center experience. Eur Rev Med Pharmacol Sci. 2022;26(10):3487-92. DOI:10.26355/eurrev_202205_28843
18. Drapkina OM, Avalueva EB, Bakulin IG, et al. Vedenie patsientov s zhelezodefitsitnoi anemiei na etape okazaniia pervichnoi mediko-sanitarnoi pomoshchi. Prakticheskoe rukovodstvo. Moscow: Vidoks, 2021 (in Russian).
19. Zia A, Stanek J, Christian-Rancy M, et al. Iron deficiency and fatigue among adolescents with bleeding disorders. Am J Hematol. 2022;97(1):60-7. DOI:10.1002/ajh.2638
20. VanderMeulen H, Arya S, Nersesian S, et al. What have we learned about the patient’s experience of von Willebrand disease? A focus on women. Hematology Am Soc Hematol Educ Program. 2022;2022(1):631-6. DOI:10.1182/hematology.2022000391
21. Ragni MV, Rothenberger SD, Feldman R, et al. Recombinant von Willebrand Factor and Tranexamic Acid for Heavy Menstrual Bleeding in VWD: Randomized Crossover Trial. Lancet Haematol. 2023;10(8):e612-23. DOI:10.1016/S2352-3026(23)00119-9
22. Kiss C, Boda Z, Khayat CD, et al. Eficacy of regular prophylaxis with a plasma-derived von Willebrand factor/factor VIIIconcentrate with a 1:1 activity ratio in reducing heavy menstrual bleeding in girls/women with von Willebrand disease. AJOG Glob Rep. 2025;5(2):100501.
23. VanderMeulen H, Tang GH, Sholzberg M. Tranexamic acid for management of heavy vaginal bleeding: barriers to access and myths surrounding its use. Res Pract Thromb Haemost. 2024;8(3):e102389 DOI:10.1016/j.rpth.2024.102389
24. Committee on Adolescent Health Care; Committee on Gynecologic Practice. Committee Opinion No. 580: Von Willebrand disease in women. Obstet Gynecol. 2013;122:1368-73. DOI:10.1097/01.AOG.0000438961.38979.19
25. Winikoff R, Scully MF, Robinson KS. Women and inherited bleeding disorders – A review with a focus on key challenges for 2019. Transfus Apher Sci. 2019;58(5):613-22. DOI:10.1016/j.transci.2019.08.013
26. NICE National Institute for Health and Care Excellence. Heavy Menstrual Bleeding: Assessment and Management. Guideline 88. National Institute for Health and Care Excellence; 2021. Available at: https://www.nice.org.uk/guidance/ng88/resources/heavy-menstrual-bleeding-assessment-and-management-p.... Accessed: 5 Sept 2022.

Авторы

М.А. Виноградова*

ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России, Москва, Россия
*mary-grape@ya.ru

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Maria A. Vinogradova*

Russian Medical Academy of Continuous Professional Education, Moscow, Russia
*mary-grape@ya.ru

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