bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.25837/HAT.2018.34..2..005bloodjour-96Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL ARTICLESОБЕСПЕЧЕНИЕ ДОЛГОСРОЧНОГО СОСУДИСТОГО ДОСТУПА У БОЛЬНЫХ ГЕМОФИЛИЕЙPROVIDING OF THE LONG-TERM VASCULAR ACCESS IN HEMOPHILIA PATIENTShttps://orcid.org/0000-0001-8818-8949ГалстянГ. М.GalstyanG. M.gengalst@gmail.comhttps://orcid.org/0000-0001-7048-060XСпиринМ. В.SpirinM. V.https://orcid.org/0000-0001-7074-0926ЗозуляН. И.ZozulyaN. I.https://orcid.org/0000-0002-4143-3094ПолянскаяТ. Ю.PolyanskayaT. Yu.https://orcid.org/0000-0002-2049-850XЗоренкоВ. Ю.ZorenkoV. Yu.ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, МоскваРоссияNational Research Center for HematologyRussian FederationФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, МоскваРоссияNational Research Center for Hematology, MoscowRussian Federation201821042019632144158Copyright © Галстян Г.М., Спирин М.В., Зозуля Н.И., Полянская Т.Ю., Зоренко В.Ю., 20192019Галстян Г.М., Спирин М.В., Зозуля Н.И., Полянская Т.Ю., Зоренко В.Ю.Galstyan G.M., Spirin M.V., Zozulya N.I., Polyanskaya T.Y., Zorenko V.Y.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/96

Введение. В России отсутствуют рекомендации по обеспечению сосудистого доступа у больных гемофилией, хотя сосудистый доступ является необходимым условием лечения данного заболевания. Цель работы — анализ обеспечения больных гемофилией долговременным сосудистым доступом. Материалы и методы. Проанализированы результаты обеспечения долговременного сосудистого доступа у 11 больных гемофилией и 1 больной болезнью Виллебранда, обратившихся в службу обеспечения сосудистого доступа ФГБУ «НМИЦ гематологии» с 2014 по 2018 год. Результаты. В общей сложности 12 больным установлены 17 устройств длительного центрального венозного доступа (УДЦВД): 11 периферически имплантируемых центральных венозных катетеров (ПИЦВК) и 6 портов. Семи больным установили 11 ПИЦВК, из них 4 больным с ингибиторной формой гемофилии, медиана длительности использования ПИЦВК составила 214 дней (от 7 до 464 дней), инцидентность катетерассоциированной инфекции кровотока (КАИК) составила 0,41 на 1000 катетеро-дней. Порты были установлены 6 больным — в 3 случаях через внутреннюю яремную вену, в 2 случаях через подключичную, у 1 больного гемофилией со стенозами и тромбозами вен бассейна верхней полой вены — в нижнюю полую путем пункции общей бедренной вены с выведением резервуара порта на переднюю поверхность бедра. Инцидентность катетерассоциированного тромбоза при использовании портов составила 0,15 на 1000 катетеро-дней, инцидентность КАИК — 0,15 на 1000 катетеро-дней. Заключение. У больных гемофилией ПИЦВК может являться альтернативой порт-системе при обеспечении длительного сосудистого доступа — прежде всего у больных ингибиторной формой гемофилии, а также если больные гемофилией нуждаются в ином лечении, нежели введение факторов свертывания. Необходимо определить потребность в УДЦВД больных гемофилией в Российской Федерации и разработать национальные рекомендации по их применению.

Background. There are no clinical guidelines in Russia regarding the use of central venous access devices in hemophilia despite venous access being crucial for hemophilia treatment. Objective. To analyse different long-term vascular access options in hemophilia patients. Materials and methods. We reviewed 12 cases (11 hemophilia patients and 1 patient with von Willebrand disease) in which long-term vascular access was established. All patients were treated in the National Research Center for Hematology between 2014 and 2018. Results. In total, 17 long-term central venous devices (LTCVD) were implanted in 12 patients (11 peripherally inserted central catheters, PICCs, and 6 ports). The PICCs were implanted in 7 patients of whom 4 had FVIII inhibitors. Median PICC dwell time was 214 days (7 to 464 days); the incidence of catheter-associated bloodstream infections was 0.41 per 1000 PICC days. The ports were implanted in 6 patients (3 via internal jugular veins, 2 via subclavian veins, and 1 via femoral vein in a patient with stenosis and thrombosis of the superior vena cava system). The incidence of catheter-associated thrombosis in this group was 0.15 per 1000 port days, and the incidence of catheter-associated bloodstream infections was 0.15 per 1000 port days. Conclusion. In inhibitor hemophilia patients and hemophilia patients who need intravenous therapy other than with clotting factors, PICCs are a good choice. It is necessary to estimate the demand for LTCVDs in hemophilia patients in Russia and to develop national guidelines regarding their use.

гемофилияболезнь Виллебрандапортпериферически имплантируемый центральный венозный катетерустройство длительного сосудистого доступаhemophiliavon Willebrand diseaseportperipherally inserted central catheterlong-term vascular accessReferencesЯгудина Р. И., Молчанова Н. Б. Обзор рынка лекарственных препаратов, применяемых при лечении гемофилии в рамках федеральной программы «7 нозологий». Фармакоэкономика: теория и практика 2016; 4:109—114.Yagudina R. I., Molchanova N. B. Market review of medicines used in the treatment of hemophilia under the federal program “7 nosologies”. Pharmacoeconomics: theory and practice (Farmakoekonomika: teoria i praktika) 2016; 4:109—114 (in Russian).Румянцев А. Г., Румянцев С. А., Чернов В. М. Гемофилия в практике врачей различных специальностей. — М.: Гэотар-Медиа, 2013.Rumyantsev A. G., Rumyantsev S. A., Chernov V. M. Hemophilia in practice of doctors of various specialties (Gemofilia v praktike vrachei razlichnyh spetsialnostei). — M.: GEOTAR-Media, 2013 (in Russian).Зозуля Н. И., Андреева Т. А., Вдовин В. В., Перина Ф. Г. Регистр пациентов с ингибиторной формой гемофилии в Российской Федерации. Актуальные вопросы трансфузиологии и клинической медицины 2015; 1:186—188.Zozulya N. I., Andreeva T. A., Vdovin V. V., Perina F. G. The register of inhibitor hemophilia patients in Russian Federation. Actual questions of transfusiology and clinical medicine (Aktualnye voprosy transfuziologii i klinicheskoi meditsiny) 2015; 1:186—188 (in Russian).Sharp R., Cummings M., Fielder A. et al. The catheter to vein ratio and rates of symptomatic venous thromboembolism in patients with a peripherally inserted central catheter (PICC): A prospective cohort study. Int J Nurs Studies; 2015; 52:677—685.Sharp R., Cummings M., Fielder A. et al. The catheter to vein ratio and rates of symptomatic venous thromboembolism in patients with a peripherally inserted central catheter (PICC): A prospective cohort study. Int J Nurs Studies; 2015; 52:677—685.Scott W. Central venous catheters. An overview of Food and Drug Administration activities. Surg Oncol Clin N Am 1995; 4:377—393.Scott W. Central venous catheters. An overview of Food and Drug Administration activities. Surg Oncol Clin N Am 1995; 4:377—393.Pittiruti M., Hamilton H., Biffi R. et al. ESPEN Guidelines on parenteral nutrition: central venous catheters (access, care, diagnosis and therapy of complications). Clinical Nutrition 2009; 28:365—377.Pittiruti M., Hamilton H., Biffi R. et al. ESPEN Guidelines on parenteral nutrition: central venous catheters (access, care, diagnosis and therapy of complications). Clinical Nutrition 2009; 28:365—377.Hamilton H., Bodenham A. R., ed. Central Venous Catheters. — Chichester: Wiley-Blacwell, 2009.Hamilton H., Bodenham A. R., ed. Central Venous Catheters. — Chichester: Wiley-Blacwell, 2009.Sandrucci S., Mussa B. ed. Peripherally inserted central venous catheters. — Milan, Heidelberg, New York, Dordrecht, London: SpringerVerlag Italia, 2014.Sandrucci S., Mussa B. ed. Peripherally inserted central venous catheters. — Milan, Heidelberg, New York, Dordrecht, London: SpringerVerlag Italia, 2014.Debourdeau P., Kassab Chahmi D., Le Gal G. et al. 2008 SOR guidelines for the prevention and treatment of thrombosis associated with central venous catheters in patients with cancer: Report from the working group. Annals of Oncology 2009; 20:1459—1471.Debourdeau P., Kassab Chahmi D., Le Gal G. et al. 2008 SOR guidelines for the prevention and treatment of thrombosis associated with central venous catheters in patients with cancer: Report from the working group. Annals of Oncology 2009; 20:1459—1471.Zerati A. E., Wolosker N., de Luccia N. et al. Totally implantable venous catheters: history, implantation technique and complications. J Vasc Bras 2017; 16:128—139.Zerati A. E., Wolosker N., de Luccia N. et al. Totally implantable venous catheters: history, implantation technique and complications. J Vasc Bras 2017; 16:128—139.Fonseca A., Nagel K., Decker K. et al. Central venous access device insertion and perioperative management of patients with severe hemophilia A: a local experience. Blood coagulation & fibrinolysis 2016; 27:156—159.Fonseca A., Nagel K., Decker K. et al. Central venous access device insertion and perioperative management of patients with severe hemophilia A: a local experience. Blood coagulation & fibrinolysis 2016; 27:156—159.Bertamino M., Riccardi F., Banov L. et al. Hemophilia care in the pediatric age. Journal of Clinical Medicine 2017; 6:54.Bertamino M., Riccardi F., Banov L. et al. Hemophilia care in the pediatric age. Journal of Clinical Medicine 2017; 6:54.Thom K. E., Holzenbein T., Jones N. et al. Arteriovenous shunts as venous access in children with Hemophilia. Hemophilia 2018; 24:429—435.Thom K. E., Holzenbein T., Jones N. et al. Arteriovenous shunts as venous access in children with Hemophilia. Hemophilia 2018; 24:429—435.Santagostino E., Gringeri A., Berardinelli L. et al. Long-term safety and feasibility of arteriovenous fistulae as vascular accesses in children with Hemophilia: a prospective study. Br J Haematol 2003; 123:502—506.Santagostino E., Gringeri A., Berardinelli L. et al. Long-term safety and feasibility of arteriovenous fistulae as vascular accesses in children with Hemophilia: a prospective study. Br J Haematol 2003; 123:502—506.Mancuso M. E., Berardinelli L. Arteriovenous fistula as stable venous access in children with severe hemophilia. Hemophilia 2010; 16:25—28.Mancuso M. E., Berardinelli L. Arteriovenous fistula as stable venous access in children with severe hemophilia. Hemophilia 2010; 16:25—28.Mancuso M. E., Berardinelli L., Beretta C. et al. Improved treatment feasibility in children with hemophilia using arteriovenous fistulae: the results after seven years of follow-up. Haematologica 2009; 94:687—692.Mancuso M. E., Berardinelli L., Beretta C. et al. Improved treatment feasibility in children with hemophilia using arteriovenous fistulae: the results after seven years of follow-up. Haematologica 2009; 94:687—692.McCarthy W. J., Valentino L. A., Bonilla A. S. et al. Arteriovenous fistula for long-term venous access for boys with hemophilia. J Vasc Surg 2007; 45:986—990.McCarthy W. J., Valentino L. A., Bonilla A. S. et al. Arteriovenous fistula for long-term venous access for boys with hemophilia. J Vasc Surg 2007; 45:986—990.Kulkarni R., Presley R. J., Lusher J. M. et al. Complications of Hemophilia in babies (first two years of life): a report from the Centers for Disease Control and Prevention Universal Data Collection System. Hemophilia 2017; 23:207—214.Kulkarni R., Presley R. J., Lusher J. M. et al. Complications of Hemophilia in babies (first two years of life): a report from the Centers for Disease Control and Prevention Universal Data Collection System. Hemophilia 2017; 23:207—214.Ewenstein B. M., Valentino L. A., Journeycake J. M. et al. Consensus recommendations for use of central venous access devices in hemophilia. Hemophilia 2004; 10:629—648.Ewenstein B. M., Valentino L. A., Journeycake J. M. et al. Consensus recommendations for use of central venous access devices in hemophilia. Hemophilia 2004; 10:629—648.Rodriguez V., Mancuso M. E., Warad D. et al. Central venous access device (CVAD) complications in hemophilia with inhibitors undergoing immune tolerance induction: Lessons from the international immune tolerance study. Hemophilia 2015; 21:e369—374.Rodriguez V., Mancuso M. E., Warad D. et al. Central venous access device (CVAD) complications in hemophilia with inhibitors undergoing immune tolerance induction: Lessons from the international immune tolerance study. Hemophilia 2015; 21:e369—374.Park Y. Avialability of peripheral inserted central catheters in severe hemophilia patients with inhibitors. Korean Journal of Pediatrics 2008; 41:1360—1362.Park Y. Avialability of peripheral inserted central catheters in severe hemophilia patients with inhibitors. Korean Journal of Pediatrics 2008; 41:1360—1362.Langley A. R., Stain A. M., Chan A. et al. Experience with central venous access devices (CVADs) in the Canadian hemophilia primary prophylaxis study (CHPS). Hemophilia 2015; 21:469—476.Langley A. R., Stain A. M., Chan A. et al. Experience with central venous access devices (CVADs) in the Canadian hemophilia primary prophylaxis study (CHPS). Hemophilia 2015; 21:469—476.Valentino L., Ewenstein B., Navickis R. J. et al. Central venous access devices in Hemophilia. Hemophilia 2004; 10:134—146.Valentino L., Ewenstein B., Navickis R. J. et al. Central venous access devices in Hemophilia. Hemophilia 2004; 10:134—146.Mancuso M. E., Mannucci P. M., Sartori A. et al. Feasibility of prophylaxis and immune tolerance induction regimens in haemophilic children using fully implantable central venous catheters. Br J Haematol 2008; 141:689—695.Mancuso M. E., Mannucci P. M., Sartori A. et al. Feasibility of prophylaxis and immune tolerance induction regimens in haemophilic children using fully implantable central venous catheters. Br J Haematol 2008; 141:689—695.Valentino L. A., Kawji M., Grygotis M. Venous access in the management of hemophilia. Blood Reviews; 2011; 25:11—15.Valentino L. A., Kawji M., Grygotis M. Venous access in the management of hemophilia. Blood Reviews; 2011; 25:11—15.Buckley B., Dreyfus J., Prasad M. et al. Burden of illness and costs among paediatric hemophilia patients with and without central venous access devices treated in US hospitals. Hemophilia 2018; 24:e93—102.Buckley B., Dreyfus J., Prasad M. et al. Burden of illness and costs among paediatric hemophilia patients with and without central venous access devices treated in US hospitals. Hemophilia 2018; 24:e93—102.Santagostino E., Mancuso M. E. Venous access in haemophilic children: Choice and management. Hemophilia 2010; 16:20—24.Santagostino E., Mancuso M. E. Venous access in haemophilic children: Choice and management. Hemophilia 2010; 16:20—24.Valentino L. A., Kapoor M. Central venous access devices in patients with hemophilia. Expert Review of Medical Devices 2005; 2:699—711.Valentino L. A., Kapoor M. Central venous access devices in patients with hemophilia. Expert Review of Medical Devices 2005; 2:699—711.Izzi G., Franchini M., Bonetti L. et al. The use of central venous catheters in Hemophilia patients. Hemophilia 2010; 16:29—31.Izzi G., Franchini M., Bonetti L. et al. The use of central venous catheters in Hemophilia patients. Hemophilia 2010; 16:29—31.Miller K., Buchanan G. R., Zappa S. et al. Implantable venous access devices in children with hemophilia: a report of low infection rates. J Pediatr 1998; 132:934—948.Miller K., Buchanan G. R., Zappa S. et al. Implantable venous access devices in children with hemophilia: a report of low infection rates. J Pediatr 1998; 132:934—948.Damiano M. L., Hutter J. J., Group T tri-regional nursing. Immune tolerance for Hemophilia patients with inhibitors: analysis of the western United States experience. Hemophilia 2000; 6:526—532.Damiano M. L., Hutter J. J., Group T tri-regional nursing. Immune tolerance for Hemophilia patients with inhibitors: analysis of the western United States experience. Hemophilia 2000; 6:526—532.Morado M., Jimenez-Yuste V., Villar A. et al. Complications of central venous catheters in patients with hemophilia and inhibitors. Hemophilia 2001; 7:551—556.Morado M., Jimenez-Yuste V., Villar A. et al. Complications of central venous catheters in patients with hemophilia and inhibitors. Hemophilia 2001; 7:551—556.Bollard C. M., Teague L., Berry E. W. et al. The use of central venous catheters (portacaths) in children with hemophilia. Hemophilia 2000; 6:66—70.Bollard C. M., Teague L., Berry E. W. et al. The use of central venous catheters (portacaths) in children with hemophilia. Hemophilia 2000; 6:66—70.Guiffant G., Durussel J. J., Flaud P. et al. Flushing ports of totally implantable venous access devices, and impact of the Huber point needle bevel orientation: Experimental tests and numerical computation. Medical Devices: Evidence and Research 2012; 5:31—37.Guiffant G., Durussel J. J., Flaud P. et al. Flushing ports of totally implantable venous access devices, and impact of the Huber point needle bevel orientation: Experimental tests and numerical computation. Medical Devices: Evidence and Research 2012; 5:31—37.Goossens G. A. Flushing and locking of venous catheters: available evidence and evidence deficit. Nurs Res Pract 2015; 2015:985686.Goossens G. A. Flushing and locking of venous catheters: available evidence and evidence deficit. Nurs Res Pract 2015; 2015:985686.Khair K., Ranta S., Thomas A. et al. The impact of clinical practice on the outcome of central venous access devices in children with hemophilia. Hemophilia 2017; 23:e276—281.Khair K., Ranta S., Thomas A. et al. The impact of clinical practice on the outcome of central venous access devices in children with hemophilia. Hemophilia 2017; 23:e276—281.Price V. E., Carcao M., Connolly B. et al. A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia. J Thromb Haemost 2004; 2:737—242.Price V. E., Carcao M., Connolly B. et al. A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia. J Thromb Haemost 2004; 2:737—242.Journeycake J. M., Quinn C. T., Miller K. L. et al. Catheter-related deep venous thrombosis in children with hemophilia. Blood 2001; 98:1727—1731.Journeycake J. M., Quinn C. T., Miller K. L. et al. Catheter-related deep venous thrombosis in children with hemophilia. Blood 2001; 98:1727—1731.Ng F., Mastoroudes H., Paul E. et al. A comparison of Hickman lineand Port-a-Cath-associated complications in patients with solid tumours undergoing chemotherapy. Clin Oncol 2007; 19:551—556.Ng F., Mastoroudes H., Paul E. et al. A comparison of Hickman lineand Port-a-Cath-associated complications in patients with solid tumours undergoing chemotherapy. Clin Oncol 2007; 19:551—556.Verso M., Agnelli G. Venous thromboembolism associated with longterm use of central venous catheters in cancer patients. J Clin Oncol 2003; 21:3665—3675.Verso M., Agnelli G. Venous thromboembolism associated with longterm use of central venous catheters in cancer patients. J Clin Oncol 2003; 21:3665—3675.Dargaud Y., Meunier S., Negrier C. Hemophilia and thrombophilia: an unexpected association! Hemophilia 2004; 10:319—326.Dargaud Y., Meunier S., Negrier C. Hemophilia and thrombophilia: an unexpected association! Hemophilia 2004; 10:319—326.Negrier C., Vial J., Vinciguerra C. et al. Combined factor IX and protein C deficiency in a child: Thrombogenic effects of two factor IX concentrates. Am J Hematol 1995; 48:120—124.Negrier C., Vial J., Vinciguerra C. et al. Combined factor IX and protein C deficiency in a child: Thrombogenic effects of two factor IX concentrates. Am J Hematol 1995; 48:120—124.Girolami A., Scandellari R., Zanon E. et al. Non-catheter associated venous thrombosis in hemophilia A and B. A critical review of all reported cases. J Thromb Thrombolysis 2006; 21:279—284.Girolami A., Scandellari R., Zanon E. et al. Non-catheter associated venous thrombosis in hemophilia A and B. A critical review of all reported cases. J Thromb Thrombolysis 2006; 21:279—284.Vepsalainen K., Lassila R., Arola M. et al. Complications associated with central venous access device in children with hemophilia: A nationwide multicentre study in Finland. Hemophilia 2015; 21:747—753.Vepsalainen K., Lassila R., Arola M. et al. Complications associated with central venous access device in children with hemophilia: A nationwide multicentre study in Finland. Hemophilia 2015; 21:747—753.Sakai T., Kohda K., Konuma Y. et al. A role for peripherally inserted central venous catheters in the prevention of catheter-related blood stream infections in patients with hematological malignancies. Int J Hematol 2014; 100:592—598.Sakai T., Kohda K., Konuma Y. et al. A role for peripherally inserted central venous catheters in the prevention of catheter-related blood stream infections in patients with hematological malignancies. Int J Hematol 2014; 100:592—598.Dougherty L., Lister S., ed. The Royal Marsden manual of clinical nursing procedures. 9th edition. — Wiley-Blacwell, 2015.Dougherty L., Lister S., ed. The Royal Marsden manual of clinical nursing procedures. 9th edition. — Wiley-Blacwell, 2015.Luft D., Schmoor C., Wilson C. et al. Central venous catheter-associated bloodstream infection and colonisation of insertion site and catheter tip. What are the rates and risk factors in haematology patients? Ann Hematol 2010; 89:1265—1275.Luft D., Schmoor C., Wilson C. et al. Central venous catheter-associated bloodstream infection and colonisation of insertion site and catheter tip. What are the rates and risk factors in haematology patients? Ann Hematol 2010; 89:1265—1275.

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