OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press11110.17650/1818-8346-2014-9-2-20-28HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CAREГЕМОБЛАСТОЗЫ: ДИАГНОСТИКА, ЛЕЧЕНИЕ, СОПРОВОДИТЕЛЬНАЯ ТЕРАПИЯNational clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuriaНациональные клинические рекомендации по диагностике и лечению пароксизмальной ночной гемоглобинурииKulaginA. D.КулагинА. Д.
Russian Federation
kulagingem@rambler.ruLisukovI. A.ЛисуковИ. А.
Russian Federation
PtushkinV. V.ПтушкинВ. В.
Russian Federation
ShilovaYe. R.ШиловаЕ. Р.
Russian Federation
TsvetaevaN. V.ЦветаеваН. В.
Russian Federation
MikhailovaYe. A.МихайловаЕ. А.
Russian Federation
First I.P. Pavlov State Medical University of St.‑Petersburg, Ministry of Health of RussiaГБОУ ВПО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И. П. Павлова» Минздрава РоссииI.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint PetersburgГБОУ ВПО «Северо-Западный государственный медицинский университет им. И. И. Мечникова» Минздрава России, Санкт-ПетербургFederal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia, MoscowФГБУ «Федеральный научно-клинический центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России, МоскваRussian Research Institute of Hematology and Transfusiology, Russian Federal Biomedical Agency, Saint PetersburgФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии» ФМБА России, Санкт-ПетербургHematology Research Center, Ministry of Health of Russia, MoscowФГБУ «Гематологический научный центр» Минздрава России, Москва170920149220281709201417092014https://oncohematology.abvpress.ru/ongm/article/view/111

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal blood disorder caused by somatic mutation of the phosphatidylinositolglycan complementation group A gene (PIG-A) in a hematopoietic stem cell. PNH is characterized by chronic intravascular hemolysis, bone marrow failure, thrombosis, renal dysfunction and other severe clinical syndromes. These clinical guidelines include definition, classification, methods and diagnostic criteria of PNH, indications for screening, optimal treatment including targeted therapy with eculizumab, bone marrow transplantation and symptomatic therapy.

Пароксизмальная ночная гемоглобинурия (ПНГ) является редким приобретенным клональным заболеванием крови, развивающимся в результате соматической мутации гена фосфатидилинозитол гликана класса А (PIG-A) в стволовых кроветворных клетках. ПНГ характеризуется хроническим внутрисосудистым гемолизом, костномозговой недостаточностью, тромбозами, нарушением функции почек и другими тяжелыми клиническими синдромами. Настоящие клинические рекомендации включают определение, классификацию, методы и критерии диагностики ПНГ, показания для скрининга, оптимальную терапевтическую тактику с включением таргетного препарата экулизумаба, трансплантации костного мозга и симптоматической терапии.

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