akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2313-7347/ob.gyn.rep.2026.726akusherstvo-2712Research ArticleREVIEW ARTICLESНАУЧНЫЕ ОБЗОРЫParoxysmal nocturnal hemoglobinuria and thrombosisПароксизмальная ночная гемоглобинурия и тромбозыhttps://orcid.org/0009-0003-5190-4958ХисамиеваА. Р.KhisamievaA. R.

Хисамиева Азалия Рустемовна

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Azaliia R. Khisamieva

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0009-0006-1782-1966ОсколковаА. Р.OskolkovaA. R.

Осколкова Арина Романовна

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Arina R. Oskolkova

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0001-8404-1042БицадзеВ. О.BitsadzeV. O.

Бицадзе Виктория Омаровна - д.м.н., проф., профессор РАН.

Scopus Author ID: 6506003478

WoS ResearcherID: F-8409-2017

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Victoria O. Bitsadze - MD, Dr Sci Med, Prof., Professor of RAS.

Scopus Author ID: 6506003478

WoS ResearcherID: F-8409-2017

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0002-0725-9686ХизроеваД. Х.KhizroevaJ. Kh.

Хизроева Джамиля Хизриевна - д.м.н., проф.

Scopus Author ID: 57194547147

WoS ResearcherID: F-8384-2017

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Djamilya Kh. Khizroeva - MD, Dr Sci Med, Prof.

Scopus Author ID: 57194547147

WoS ResearcherID: F-8384-2017

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0002-2541-3843МакацарияН. А.MakatsariyaN. A.

Макацария Наталия Александровна - к.м.н.

WoS ResearcherID: F-84062017

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Nataliya A. Makatsariya - MD, PhD.

WoS ResearcherID: F-84062017

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0002-3628-0804ТретьяковаМ. В.TretyakovaM. V.

Третьякова Мария Владимировна - к.м.н.

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Maria V. Tretyakova - MD, PhD.

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0003-0764-4477ГашимоваН. Р.GashimovaN. R.

Гашимова Нилуфар Рамиль кызы - к.м.н.

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Nilufar R. Gashimova - MD, PhD.

8 bldg. 2, Trubetskaya Str., Moscow 119048

nelya.94@yandex.ruhttps://orcid.org/0000-0002-7756-8935ГригорьеваК. Н.GrigorevaK. N.

Григорьева Кристина Николаевна - к.м.н.

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Kristina N. Grigoreva - MD, PhD.

8 bldg. 2, Trubetskaya Str., Moscow 119048

https://orcid.org/0000-0002-3367-9844БлиновД. В.BlinovD. V.

Блинов Дмитрий Владиславович – д.м.н.

Scopus Author ID: 6701744871

WoS ResearcherID: E-8906-2017

eLibrary SPIN-code: 9779-8290

101000 Москва, Лялин переулок, д. 11–13/1; 123056 Москва, 2-я Брестская ул., д. 5, с. 1–1а; Московская область, деревня Голубое, ул. Родниковая, стр. 6, к. 1

Dmitry V. Blinov - MD, Dr Sci Med.

Scopus Author ID: 6701744871

WoS ResearcherID: E-8906-2017

eLibrary SPIN-code: 9779-8290

11–13/1 Lyalin Pereulok, Moscow 101000; 5 bldg. 1–1a, 2-ya Brestskaya Str., Moscow 123056; 6 bldg. 1, Rodnikovaya Str., Village Goluboe, Moscow region 141551

https://orcid.org/0000-0002-9899-9910ГриЖ.-К.GrisJ.-Ch.

Гри Жан-Кристоф - д.м.н., проф., иностранный член РАН.

Scopus Author ID: 7005114260

WoS ResearcherID: AAA-2923-2019

119048 Москва, ул. Трубецкая, д. 8, стр. 2; Франция, 34090 Монпелье, ул. Огюста Бруссоне, д. 163

Jean-Christophe Gris - MD, Dr Sci Med, Prof., Foreign Member of RAS.

Scopus Author ID: 7005114260

WoS ResearcherID: AAA-2923-2019

8 bldg. 2, Trubetskaya Str., Moscow 119048; 163 Rue Auguste Broussonnet, Montpellier 34090

https://orcid.org/0000-0002-9576-1368ЭлаламиИ.ElalamyI.

Элалами Исмаил - д.м.н., проф., иностранный член РАН.

Scopus Author ID: 7003652413

WoS ResearcherID: AAC-9695-2019

119048 Москва, ул. Трубецкая, д. 8, стр. 2; Марокко, 42312 Марракеш, шоссе Амизмиз, 13-й км; 92200 Париж, Neuilly-sur-Seine, улица дю Шато, д. 55

Ismail Elalamy - MD, Dr Sci Med, Prof., Foreign Member of RAS.

Scopus Author ID: 7003652413

WoS ResearcherID: AAC-9695-2019

8 bldg. 2, Trubetskaya Str., Moscow 119048; Km 13, Route d’Amizmiz, Marrakech 42312, Morocco; 55 Rue du Châtea; Neuilly-sur-Seine, Paris 92200

https://orcid.org/0000-0003-2316-6348ГеротзиафасГ.GerotziafasG.

Геротзиафас Григориос - д.м.н., проф., иностранный член РАН.

119048 Москва, ул. Трубецкая, д. 8, стр. 2; 75020 Париж, Китайская улица, д. 4; 75013 Париж, Бульвар Госпитале, д. 91; 75012 Париж, улица дю Фобур Сен-Антуан, д. 184

Grigoriоs Gerotziafas - MD, Dr Sci Med, Prof., Foreign Member of RAS.

8 bldg. 2, Trubetskaya Str., Moscow 119048; 4 Rue de la Chine, 75020 Paris; 91 Boulevard de l'Hôpital, 75013 Paris; 34 Rue du Crozatier, F-75012 Paris

https://orcid.org/0000-0001-7415-4633МакацарияА. Д.MakatsariyaA. D.

Макацария Александр Давидович - д.м.н., проф., академик РАН.

Scopus Author ID: 57222220144

WoS ResearcherID: M-5660-2016

119048 Москва, ул. Трубецкая, д. 8, стр. 2

Alexander D. Makatsariya - MD, Dr Sci Med, Prof., Academician of RAS.

Scopus Author ID: 57222220144

WoS ResearcherID: M-5660-2016

8 bldg. 2, Trubetskaya Str., Moscow 119048

ФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет)РоссияSechenov UniversityRussian FederationИнститут Превентивной и Социальной Медицины; АНО ДПО «Московский медико-социальный институт имени Ф.П. Гааза»; ФГБУ «Федеральный научно-клинический центр медицинской реабилитации и курортологии Федерального медико-биологического агентства»РоссияInstitute for Preventive and Social Medicine; Moscow Haass Medical – Social Institute; Federal Scientific and Clinical Center for Medical Rehabilitation and Balneology, Federal Medical-Biological AgencyRussian FederationФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет); Университет МонпельеРоссияSechenov University; University of MontpellierRussian FederationФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет); Частный университет Марракеша; Американский госпиталь ПарижаРоссияSechenov University; Université Privée de Marrakech; Hopital Americain de ParisRussian FederationФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет); Госпиталь Тенон, Университетский институт онкологии AP-HP, Университет Сорбонна; Медицинский факультет Сорбонского университета; Университетская больница Сен-Антуан, Университетский институт онкологии AP-HP, Университет СорбоннаФранцияSechenov University; Tenon Hospital, Hôpitaux Universitaires Est Parisien, Assistance Publique Hôpitaux de Paris (AP-HP), France; Faculty of Medicine Sorbonne University; Saint-Antoine Research Center (CRSA), University Institute of Cancerology (UIC)France202612032026201146157Copyright © Khisamieva A.R., Oskolkova A.R., Bitsadze V.O., Khizroeva J.K., Makatsariya N.A., Tretyakova M.V., Gashimova N.R., Grigoreva K.N., Blinov D.V., Gris J., Elalamy I., Gerotziafas G., Makatsariya A.D., 20262026Хисамиева А.Р., Осколкова А.Р., Бицадзе В.О., Хизроева Д.Х., Макацария Н.А., Третьякова М.В., Гашимова Н.Р., Григорьева К.Н., Блинов Д.В., Гри Ж., Элалами И., Геротзиафас Г., Макацария А.Д.Khisamieva A.R., Oskolkova A.R., Bitsadze V.O., Khizroeva J.K., Makatsariya N.A., Tretyakova M.V., Gashimova N.R., Grigoreva K.N., Blinov D.V., Gris J., Elalamy I., Gerotziafas G., Makatsariya A.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gynecology.su/jour/article/view/2712

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal hematopoiesis disorder caused by a somatic mutation in the PIGA (phosphatidylinositol glycan, class A) gene, resulting in deficiency of glycosylphosphatidylinositol (GPI)anchored complement regulatory proteins on blood cell surface. Dysregulation of the complement system leads to chronic intravascular hemolysis, anemia, endothelial dysfunction as well as development of prominent acquired thrombophilic state, clinically manifested by venous thromboembolism, frequently involving atypical vascular sites. Thrombotic complications represent the leading cause of adverse outcomes and mortality in patients with PNH. This review summarizes current evidence on PNH etiology, molecular genetics and pathophysiological mechanisms including complement dysregulation, nitric oxide depletion, intraand extravascular hemolysis, and the multifactorial pathogenesis of thrombosis. The clinicopathogenetic relationship between PNH, aplastic anemia, and myelodysplastic syndromes is discussed in the context of immune-mediated bone marrow failure. Special emphasis is put on PNH course in women of reproductive age. Physiological changes associated with pregnancy and the postpartum period, including complement activation and hypercoagulability, are shown to be associated with increased risk of hemolytic crises and thromboembolic complications in women with PNH, thereby defining the disease as a condition of extremely high obstetric risk. Contemporary principles for multidisciplinary management aimed at improving maternal and perinatal outcomes are reviewed.

Пароксизмальная ночная гемоглобинурия (ПНГ) является редким приобретенным клональным заболеванием системы кроветворения, обусловленным соматической мутацией гена PIGA (англ. phosphatidylinositol glycan, class A; фосфатидилинозитолгликан класса А) и дефицитом гликозилфосфатидилинозитол (англ. glycosylphosphatidylinositol, GPI)-связанных регуляторных белков комплемента на поверхности клеток крови. Дисрегуляция системы комплемента приводит к хроническому внутрисосудистому гемолизу, анемии, эндотелиальной дисфункции и формированию выраженного приобретенного тромбофилического состояния, клинически проявляющегося венозными тромбозами, в том числе атипичной локализации. Тромботические осложнения являются ведущей причиной неблагоприятного прогноза и летальности при ПНГ. В статье обобщены современные данные об этиологии, молекулярно-генетических и патофизиологических механизмах ПНГ, включая нарушения регуляции системы комплемента, дефицит оксида азота, экстраи внутрисосудистый гемолиз, а также клинико-патогенетическую связь заболевания с апластической анемией и миелодиспластическими синдромами. Особое внимание уделено течению ПНГ у женщин репродуктивного возраста. Показано, что физиологические изменения беременности и послеродового периода, включая активацию системы комплемента и гиперкоагуляцию, у пациенток с ПНГ ассоциированы с повышенным риском гемолитических кризов и тромбоэмболических осложнений, что позволяет рассматривать данное заболевание как состояние крайне высокого акушерского риска. Рассмотрены современные принципы междисциплинарного ведения пациенток, направленные на улучшение материнских и перинатальных исходов.

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The authors declare that there are no conflicts of interest present.