akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2313-7347/ob.gyn.rep.2022.288akusherstvo-1313Research ArticleCLINICAL CASEКЛИНИЧЕСКИЙ СЛУЧАЙMasked polycythemia vera manifested by splanchnic venous thrombosis during pregnancy: a clinical caseМаскированная истинная полицитемия, манифестировавшая спланхническим венозным тромбозом во время беременности: клинический случайhttps://orcid.org/0000-0002-1210-2528НадинскаяМ. Ю.NadinskaiaM. Yu.

к.м.н., доцент кафедры пропедевтики внутренних болезней, гастроэнтерологии и гепатологии Института клиническоймедицины имени Н.В. Склифосовского

Scopus Author ID: 6507949442. Researcher ID: T-9316-2019

Россия, 119991 Москва, ул. Трубецкая, д. 8, стр. 2

MD, PhD, Associate Professor, Department of Propaedeutics of Internal Diseases, Gastroenterology and Hepatology, Sklifosovsky Institute of Clinical Medicine

Scopus Author ID: 6507949442. Researcher ID: T-9316-2019

 8 bldg. 2, Trubetskaya Str., Moscow 119991, Russia

nadinskaya_m_yu@staff.sechenov.ruhttps://orcid.org/0000-0001-7510-6553КодзоеваХ. Б.KodzoevaKh. B.

аспирант кафедры пропедевтики внутренних болезней, гастроэнтерологии и гепатологии Института клиническоймедицины имени Н.В. Склифосовского

Scopus Author ID: 57208035765. Researcher ID: P-6999-2018

Россия, 119991 Москва, ул. Трубецкая, д. 8, стр. 2

 MD, Postgraduate Student, Department of Propaedeutics of Internal Diseases, Gastroenterology and Hepatology, Sklifosovsky Institute of Clinical Medicine

Scopus Author ID: 57208035765. Researcher ID: P-6999-2018

 8 bldg. 2, Trubetskaya Str., Moscow 119991, Russia

https://orcid.org/0000-0003-1682-7005СоколоваМ. А.SokolovaM. A.

к.м.н., старший научный сотрудник консультативного гематологического отделения с дневным стационаром попроведению интенсивной высокодозной химиотерапии

Scopus Author ID: 7101745419. Researcher ID: N-4843-2014

Россия, 125167 Москва, Новый Зыковский проезд, д. 4

MD, PhD, Senior Researcher, Consultative Hematology Department with a Day Hospital for Intensive High-Dose Chemotherapy

Scopus Author ID: 7101745419. Researcher ID: N-4843-2014

4 Novyi Zykovskiy Рroezd, Moscow 125167, Russia

https://orcid.org/0000-0002-7310-974XАпресянС. В.ApresyanS. V.

д.м.н., профессор кафедры женских болезней и репродуктивного здоровья института усовершенствования врачей; зам. главного врача по акушерско-гинекологической помощи

Scopus Author ID: 57208050125

Россия, 105203 Москва, ул. Нижняя Первомайская, д. 70

Россия, 129327 Москва, ул. Ленская, д. 15

MD, Dr Sci Med, Professor, Department of Women's Diseases and Reproductive Health, Institute for Postgraduate Medical Education; Deputy Chief Physician for Obstetric and Gynecological Care

Scopus Author ID: 57208050125

70 Nizhnyaya Pervomaiskaya Str., Moscow 105203, Russia

15 Lenskaya Str., Moscow 129327, Russia

https://orcid.org/0000-0001-9862-8266КрыжановскаяЕ. Ю.KryzhanovskayaE. Yu.

к.м.н., старший научный сотрудник отдела клинической физиологии, инструментальной и лучевой диагностики

Scopus Author ID: 57192997015

Россия, 119991 Москва, Абрикосовский пер., д. 2

MD, PhD, Senior Researcher, Department of Clinical Physiology, Instrumental and Radiation Diagnostics

Scopus Author ID: 57192997015

2 Abrikosovsky Lane, Moscow 119991, Russia

https://orcid.org/0000-0001-9349-8327ЩукинаА. А.ShchukinaA. A.

аспирант, лаборатория экстренной хирургии и портальной гипертензии 

Россия, 119991 Москва, Абрикосовский пер., д. 2

MD, Postgraduate Student, Laboratory of Emergency Surgery and Portal Hypertension

2 Abrikosovsky Lane, Moscow 119991, Russia

https://orcid.org/0000-0002-8268-3129КиценкоЕ. А.KitsenkoE. A.

д.м.н., ведущий научный сотрудник лаборатории экстренной хирургии и портальной гипертензии

Scopus Author ID: 6602462726

Россия, 119991 Москва, Абрикосовский пер., д. 2

MD, Dr Sci Med, Leading Researcher, Laboratory of Emergency Surgery and Portal Hypertension

Scopus Author ID: 6602462726

2 Abrikosovsky Lane, Moscow 119991, Russia

ФГАОУ ВО Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский Университет)РоссияSechenov UniversityRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Research Center for HematologyRussian FederationФГБУ «Национальный медико-хирургический центр имени Н.И. Пирогова» Министерства здравоохранения Российской Федерации; ГБУЗ «Городская клиническая больница имени А.К. Ерамишанцева Департамента здравоохранения города Москвы»РоссияPirogov National Medical and Surgical Center; Eramishantsev City Clinical HospitalRussian FederationФГБНУ «Российский научный центр хирургии имени академика Б.В. Петровского»РоссияPetrovsky National Research Centre of SurgeryRussian Federation202214052022162194203Copyright © Nadinskaia M.Y., Kodzoeva K.B., Sokolova M.A., Apresyan S.V., Kryzhanovskaya E.Y., Shchukina A.A., Kitsenko E.A., 20222022Надинская М.Ю., Кодзоева Х.Б., Соколова М.А., Апресян С.В., Крыжановская Е.Ю., Щукина А.А., Киценко Е.А.Nadinskaia M.Y., Kodzoeva K.B., Sokolova M.A., Apresyan S.V., Kryzhanovskaya E.Y., Shchukina A.A., Kitsenko E.A.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/1313

Ph-negative myeloproliferative disorders such as polycythemia vera (PV), essential thrombocythemia, and primary myelofibrosis are rare clonal hematopoietic diseases that typically occur at age over 50. Only 10–15% of PV cases are diagnosed during the reproductive years, most commonly manifested as splanchnic venous thrombosis (SVT). Here, we demonstrate a case of a 6-weeks pregnant woman with a history of stillbirth and miscarriage presented with SVT. A terminal ileal resection and right hemicolectomy were performed, as well as the appointment of low molecular weight heparin in the postoperative period was initiated that allowed the patient to continue the pregnancy and deliver a child. The JAK2 V617F mutation was detected and masked PV was diagnosed according to bone marrow findings. In this article, we discuss the etiology of SVT in adults and effect of JAK2 V617F mutation on pregnancy outcomes as well as summarize current knowledge about pregnancy outcomes in PV.

Ph-негативные миелопролиферативные заболевания – истинная полицитемия (ИП), эссенциальная тромбоцитемия, первичный миелофиброз – редкие клональные заболевания системы крови, развивающиеся в основном у лиц старше 50 лет. В репродуктивном возрасте диагностируется до 10–15 % случаев ИП, характерной особенностью дебюта служит развитие спланхнического венозного тромбоза (СВТ). Представлен случай пациентки с мертворождением и самопроизвольным абортом в анамнезе, у которой во время третьей беременности на сроке 6 нед развился СВТ. Выполненная резекция терминального отдела подвздошной кишки и правосторонняя гемиколэктомия, а также назначение в послеоперационном периоде низкомолекулярного гепарина позволили пролонгировать беременность и обеспечить её благоприятный исход. Обнаружена мутация V617F гена JAK2, пациентка консультирована гематологом, проведена трепанобиопсия, по результатам которой диагностирована ИП (маскированная форма). Обсуждаются причины развития СВТ у взрослых, влияние мутации JAK2 V617F на исходы беременности; проведен анализ исходов ИП у беременных.

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