bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.18821/0234-5730-2016-61-1-49-53bloodjour-14Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESОсобенности ведения пациентов с болезнью Виллебранда: опыт Санкт-Петербургского Городского центра по лечению гемофилииManagement of patients with von Willebrand disease: practice of St Petersburg City Hemophilia Care Centrehttps://orcid.org/0000-0002-5649-325XАндрееваТ. А.AndreevaT. A.

Андреева Татьяна Андреевна- кандидат медицинских наук, заведующая

191186, г. Санкт-Петербург

Andreeva Tatiana А., MD, PhD, head of City Hemophilia Care Centre, City Outpatient Medical Clinic No37

191186, St. Petersburg

spbhc@msn.comЛавриченкоИ. А.LavrichenkoI. A.

191186, г. Санкт-Петербург

191186, St. Petersburg

КлимоваН. И.KlimovaN. I.

191186, г. Санкт-Петербург

191186, St. Petersburg

https://orcid.org/0000-0001-8813-1272ЗалепухинаО. Э.ZalepukhinaO. E.

191186, г. Санкт-Петербург

191186, St. Petersburg

КонстантиноваВ. Н.KonstantinovaV. N.

191186, г. Санкт-Петербург

191186, St. Petersburg

https://orcid.org/0000-0002-3422-5043КимА. В.KimA. V.

191186, г. Санкт-Петербург

191186, St. Petersburg

Городской центр по лечению гемофилии Санкт-Петербургского ГБУЗ «Городская поликлиника №37»РоссияCity Hemophilia Care CentreRussian Federation2016250220196114953Copyright © Андреева Т.А., Лавриченко И.А., Климова Н.И., Залепухина О.Э., Константинова В.Н., Ким А.В., 20192019Андреева Т.А., Лавриченко И.А., Климова Н.И., Залепухина О.Э., Константинова В.Н., Ким А.В.Andreeva T.A., Lavrichenko I.A., Klimova N.I., Zalepukhina O.E., Konstantinova V.N., Kim A.V.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/14

Болезнь Виллебранда (БВ) – наследственное заболевание, передающееся аутосомно-доминантным или аутосомно-рецессивным путем, обусловленное различными количественными и качественными дефектами синтеза фактора Виллебранда (ФВ) и характеризующееся симптомами повышенной кровоточивости. В России2013 г. был объявлен годом болезни Виллебранда. C целью разработки рекомендаций по совершенствованию специализированной медицинской помощи детям и взрослым с БВ в России в рамках данного мероприятия было проведено ретроспективное исследование со сбором информации о больных, состоящих на учете у гематологов регионов с диагнозом БВ (код по МКБ-10 D 68.0). Было обследовано 86 пациентов с подозрением на БВ из регионов Российской Федерации. Диагноз был установлен или подтвержден у 52 (61,6%) пациентов. Представленные данные подтвердили, что современное состояние лабораторной службы в регионах РФ не позволяет выявлять нарушения в системе гемостаза для установления окончательного диагноза. Применение концентратов фактора свертывания VIII + ФВ представляет для России достаточно новый, пока редко применяемый терапевтический подход для остановки кровотечений у пациентов с БВ. Вместе с тем, по результатам данного исследования, он оказался эффективным во всех случаях его применения.

Von Willebrand disease (vWD) is a heritable disease, which can be inherited by either autosomal dominant or autosomal recessive, caused by qualitative or quantitative deficiency of von Willebrand factor (vWF) and characterized by excessive bleeding. In Russia 2013 was announced as a year of von Willebrand disease. For elaboration of guidelines on improvement of specific care of children and adults suffering from vWD we carried out a retrospective study. Information regarding the patients registered by regional haematologists with diagnosis of vWD (ICH-10 code D 68.0) was collected. There were examined 86 patients suspected for vWD. The diagnosis was confirmed in 52 (61.6%) patients. This data supported opinion that present status of laboratory service in the Russian regions is not satisfactory for detection of coagulation disorders and final diagnosis justification. Factor VIII concentrate is relatively novel but rare for Russian practice therapeutic approach for bleeding treatment in the patients with vWD. According to the results of the study it was effective in all the cases.

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