bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2022-67-4-535-550bloodjour-409Research ArticleОБЗОРЫREVIEWSОсобенности клинико-лабораторной диагностики редкой коагулопатии — приобретенной гемофилииFeatures of clinical and laboratory diagnosis of rare coagulopathy – acquired hemophiliahttps://orcid.org/0000-0003-2439-6559СуренковА. А.SurenkovA. A.

Суренков Алексей Алексеевич, врач централизованной клинико-диагностической лаборатории

125167, Москва

Aleksei A. Surenkov, Physician, Central Clinical Diagnostic Laboratory

125167, Moscow

aleksei_surenkov@mail.ruhttps://orcid.org/0000-0002-7932-7617ОрелЕ. Б.OrelE. B.

Орел Елена Борисовна, руководитель группы патологии гемостаза централизованной клинико-диагностической лаборатории

125167, Москва

Elena B. Orel, Head of the Hemostasis Pathology Group, Central Clinical Diagnostic Laboratory

125167, Moscow

orel.e@blood.ruhttps://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 Diagnostic Department of Hematology and Hemostasis Disorders

125167, Moscow

zozulya.n@blood.ruhttps://orcid.org/0000-0002-9877-0796ДвирныкВ. Н.DvirnykV. N.

Двирнык Валентина Николаевна, кандидат медицинских наук, заведующая централизованной клинико-диагностической лабораторией

125167, Москва

Valentina N. Dvirnyk, Cand. Sci. (Med.), Head of the Central Clinical Diagnostic Laboratory

125167, Moscow

dvirnyk.v@blood.ruФГБУ «Национальный медицинский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation202227122022674535550Copyright © Суренков А.А., Орел Е.Б., Зозуля Н.И., Двирнык В.Н., 20222022Суренков А.А., Орел Е.Б., Зозуля Н.И., Двирнык В.Н.Surenkov A.A., Orel E.B., Zozulya N.I., Dvirnyk V.N.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/409Введение

Введение. Образование циркулирующих аутоантител, способных ингибировать факторы свертывающей системы крови, сопровождается возникновением спонтанных и/или посттравматических кровотечений у больных без предшествующих нарушений системы гемостаза в анамнезе. Одной из причин развития таких состояний является приобретенная гемофилия.

Цель – представить алгоритмы лабораторной диагностики приобретенной гемофилии.

Основные сведения

Основные сведения. Первичная диагностика и контроль терапии заболевания осуществляются на основании результатов коагулологических исследований, при расшифровке и интерпретации которых часто возникают сложности, обусловленные низкой осведомленностью врачей об алгоритмах лабораторной диагностики и тактике ведения больных. При приобретенной гемофилии отсутствует прямая связь между результатами лабораторных исследований и клиническими проявлениями, что определяется кинетикой взаимодействия аутоантител с фактором свертывания крови (F) VIII. Встречается «ложное» in vitro снижение активности факторов внутреннего пути (FIX, FXI и FXII), ассоциированное с эффектом «быстродействующего» ингибитора в высоких титрах. Важной задачей представляется определение волчаночного антикоагулянта, который затрудняет своевременную диагностику и верификацию диагноза.

Introduction

Introduction. The formation of circulating autoantibodies capable of inhibiting factors of the blood coagulation system is accompanied by the occurrence of spontaneous and/or post-traumatic bleeding in patients without a history of previous disorders of the hemostasis system. One of the reasons for the development of such conditions is acquired hemophilia.

Aim – to present algorithms for laboratory diagnosis of acquired hemophilia.

Main findings

Main findings. Primary diagnosis and control of therapy of the disease are carried out based on the results of coagulation studies, the decoding and interpretation of which often causes difficulties due to the low awareness of doctors about the algorithms for laboratory diagnosis and tactics for managing patients. In acquired hemophilia there is no direct relationship between the results of laboratory tests and the clinical manifestations of the disease, which is determined by the kinetics of the interaction of autoantibodies with blood coagulation factor (F) VIII. There is a «false» in vitro decrease in the activity of factors of the internal pathway (FIX, FXI and FXII), associated with the effect of a rapid inhibitor in high titers. An important laboratory task is the determination of lupus anticoagulant, which makes it difficult to timely diagnose and verify the diagnosis.

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