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National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria
- Authors: Kulagin A.D.1, Lisukov I.A.2, Ptushkin V.V.3, Shilova Y.R.4, Tsvetaeva N.V.5, Mikhailova Y.A.5
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Affiliations:
- First I.P. Pavlov State Medical University of St.‑Petersburg, Ministry of Health of Russia
- I.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint Petersburg
- Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia, Moscow
- Russian Research Institute of Hematology and Transfusiology, Russian Federal Biomedical Agency, Saint Petersburg
- Hematology Research Center, Ministry of Health of Russia, Moscow
- Issue: Vol 9, No 2 (2014)
- Pages: 20-28
- Section: HEMATOLOGIC MALIGNANCIES: DIAGNOSIS, TREATMENT, SUPPORTIVE CARE
- Published: 17.09.2014
- URL: https://oncohematology.abvpress.ru/ongm/article/view/111
- DOI: https://doi.org/10.17650/1818-8346-2014-9-2-20-28
- ID: 111
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Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal blood disorder caused by somatic mutation of the phosphatidylinositol
glycan 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.
About the authors
A. D. Kulagin
First I.P. Pavlov State Medical University of St.‑Petersburg, Ministry of Health of Russia
Author for correspondence.
Email: kulagingem@rambler.ru
Russian Federation
I. A. Lisukov
I.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint PetersburgRussian Federation
V. V. Ptushkin
Federal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia, MoscowRussian Federation
Ye. R. Shilova
Russian Research Institute of Hematology and Transfusiology, Russian Federal Biomedical Agency, Saint PetersburgRussian Federation
N. V. Tsvetaeva
Hematology Research Center, Ministry of Health of Russia, MoscowRussian Federation
Ye. A. Mikhailova
Hematology Research Center, Ministry of Health of Russia, MoscowRussian Federation
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