bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2023-68-2-219-228bloodjour-457Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESCOVID-19 у больных наследственными коагулопатиями в РоссииCOVID-19 in patients with congenital bleeding disorders in Russiahttps://orcid.org/0000-0002-6991-7437ЯковлеваЕ. В.YakovlevaE. V.

Яковлева Елена Владимировна, кандидат медицинских наук, научный сотрудник, гематолог клинико-диагностического отделения гематологии и  нарушений гемостаза

125167, Москва

Elena V. Yakovleva, Cand. Sci. (Med.), Scientific Researcher, Hematologist, Clinical and Diagnostic Department of Hematology and Hemostasis Disorders

125167, Moscow

hemophilia2012@mail.ruhttps://orcid.org/0000-0001-7506-4947ВыборныхД. Э.VybornykhD. E.

Выборных Дмитрий Эдуардович, доктор медицинских наук, заведующий лабораторией по изучению психических и неврологических расстройств при заболеваниях системы крови

125167, Москва

Dmitry E. Vybornykh, Dr. Sci. (Med.), Head of the Laboratory for the Study of Mental and Neurological Disorders in Diseases of the Blood System

125167, Moscow

https://orcid.org/0000-0002-8357-977XГемджянЭ. Г.GemdzhianE. G.

Гемджян Эдуард Георгиевич, научный сотрудник лаборатории по изучению психических и неврологических расстройств при заболеваниях системы крови

125167, Москва

Eduard G. Gemdzhian, Scientifi c Researcher, Laboratory for the Study of Mental and Neurological Disorders in Diseases of the Blood System

125167, Moscow

https://orcid.org/0000-0001-7074-0926ЗозуляН. И.ZozulyaN. I.

Зозуля Надежда Ивановна, доктор медицинских наук, заведующая клинико-диагностическим отделением гематологии и нарушений гемостаза

125167, Москва

Nadezhda I. Zozulya, Dr. Sci. (Med.), Head of the Clinical and Diagnostic Department of Hematology and Hemostasis Disorders

125167, Moscow

ФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202320072023682219228Copyright © Яковлева Е.В., Выборных Д.Э., Гемджян Э.Г., Зозуля Н.И., 20232023Яковлева Е.В., Выборных Д.Э., Гемджян Э.Г., Зозуля Н.И.Yakovleva E.V., Vybornykh D.E., Gemdzhian E.G., Zozulya N.I.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/457Введение

Введение. Сведения о течении новой коронавирусной инфекции (COVID-19) у больных наследственными коагулопатиями ограничены. Основными вопросами являются: как наследственные коагулопатии влияют на течение COVID-19, и, наоборот, как COVID-19 влияет на течение наследственных коагулопатий.

Цель — анализ течения COVID-19 у больных наследственными коагулопатиями в России.

Методы

Методы. За период с 25.06.2022 по 31.07.2022 проведено поперечное опросное исследование, в которое были включены 187 больных наследственными коагулопатиями из разных городов и областей России. Использовали анкету на основе программного обеспечения для администрирования опросов (Google forms), включающую 27 вопросов.

Результаты

Результаты. Перенесли COVID-19 115 (62 %) из 187 опрошенных больных наследственными коагулопатиями. Дважды COVID-19 перенесли 22 (19 %) больных. Госпитализация потребовалась 14 (12 %) больным: 10 — с тяжелой гемофилией А, 1 — с гемофилией В среднетяжелой формы, 2 — с болезнью Виллебранда и 1 — с гипопроконвертинемией. Средний возраст госпитализированных больных составил 42 года. Во время COVID-19 геморрагический синдром в виде гемартрозов, экхимозов, гематом, носовых кровотечений, меноррагий, геморроидальных кровотечений, кровоизлияний в глазное яблоко отмечался у 9 (8 %) больных. Случаев тромбоза среди 115 переболевших COVID-19 не было. Среди больных наследственными коагулопатиями, переболевших COVID-19, по сравнению с больными, не болевшими COVID-19, болезнь Виллебранда встречалась статистически значимо чаще (р = 0,04). Изменения в течении основного заболевания после перенесенной COVID-19 констатировали 21 (18 %) из 115 больных: усиление болей в суставах отметили 11 (10 %) переболевших, 9 (8 %) жаловались на появление болей в суставах, которые ранее не беспокоили; изменения характера геморрагического синдрома описали 10 % больных.

Заключение

Заключение. Вероятность госпитализации больных наследственными коагулопатиями при COVID-19 старше 40 лет значимо выше. Болезнь Виллебранда может рассматриваться как потенциальный фактор риска COVID-19. Учитывая отсутствие случаев тромбозов в опрошенной группе, возможно, наличие гипокоагуляции у больных наследственными коагулопатиями является защитным механизмом, препятствующим возникновению тромботических осложнений при COVID-19.

Background

Background. Internationally published data about the course of COVID-19 in patients with congenital bleeding disorders (CBDs) are limited. There are questions about how COVID-19 affects the course of CBDs and, conversely, how CBDs affect the course of coronavirus infection.

Aim — to analyze the course of COVID-19 in patients with CBDs in Russia.

Materials and methods

Materials and methods. A cross-sectional survey was conducted at the National Medical Research Center for Hematology (Moscow) for the period from June 25 to July 31, 2022. A cluster of 187 patients from different regions and cities of Russia were interviewed with a questionnaire based on survey administration software (Google forms) containing 27 questions.

Results

Results. COVID-19 affected 115 (62 %) of 187 surveyed patients and 22 (19 %) patients suffered from coronavirus infection twice. Hospitalization was required for 14 (12 %) patients with an average age of 42 years (10 patients with severe hemophilia A, 1 patient with moderate hemophilia B, 2 patients with von Willebrand disease and 1 patient with hypoproconvertinemia). During COVID-19 bleeding was observed in 9 (8 %) patients and was represented by hemarthrosis, ecchymosis, hematomas, nosebleeds, menorrhagia, hemorrhoidal bleeding, hemorrhage in the eyeball. There were no cases of thrombosis among 115 COVID-19 patients. Among patients with CBDs who had COVID-19, compared with patients who had not COVID-19, von Willebrand disease was statistically significantly more common (p = 0.04). Changes in the course of CBD after COVID-19 were noted by 21 (18 %) of 115 patients: 11 (10 %) of those who were ill noted increased joint pain, 9 (8 %) complained of joint pain that had not been previously experienced; 10 % of patients described changes of hemorrhagic syndrome.

Conclusion

Conclusion. The probability of hospitalization of patients with CBDs and COVID-19 older than 40 is statistically significantly higher. Von Willebrand disease can be considered as a potential risk factor for COVID-19. Given the absence of cases of thrombosis in the interviewed group of patients, the results of the study suggest that the presence of hypocoagulation in patients with CBDs may be a protective pathophysiological mechanism that prevents the development of COVID-19-associated thrombotic complications.

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