National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria
<p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal blood disorder caused by somatic mutation of the phosphatidylinositol<br />glycan complementation group A gene (PIG-A) in a hematopoietic stem cell. PNH is characterized by chronic intravascular hemolysis, bone marrow...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | Russian |
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ABV-press
2014-09-01
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| Series: | Онкогематология |
| Subjects: | |
| Online Access: | http://oncohematology.abvpress.ru/index.php/ongm/article/view/111 |
| _version_ | 1811173575723319296 |
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| author | A. D. Kulagin I. A. Lisukov V. V. Ptushkin Ye. R. Shilova N. V. Tsvetaeva Ye. A. Mikhailova |
| author_facet | A. D. Kulagin I. A. Lisukov V. V. Ptushkin Ye. R. Shilova N. V. Tsvetaeva Ye. A. Mikhailova |
| author_sort | A. D. Kulagin |
| collection | DOAJ |
| description | <p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal blood disorder caused by somatic mutation of the phosphatidylinositol<br />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.</p> |
| first_indexed | 2024-04-10T17:50:17Z |
| format | Article |
| id | doaj.art-f349757c62a74939ab2532630746408a |
| institution | Directory Open Access Journal |
| issn | 1818-8346 |
| language | Russian |
| last_indexed | 2024-04-10T17:50:17Z |
| publishDate | 2014-09-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Онкогематология |
| spelling | doaj.art-f349757c62a74939ab2532630746408a2023-02-02T22:27:58ZrusABV-pressОнкогематология1818-83462014-09-01922028126National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuriaA. D. Kulagin0I. A. Lisukov1V. V. Ptushkin2Ye. R. Shilova3N. V. Tsvetaeva4Ye. A. Mikhailova5First I.P. Pavlov State Medical University of St.‑Petersburg, Ministry of Health of RussiaI.I. Mechnikov North-Western State Medical University, Ministry of Health of Russia, Saint PetersburgFederal Research Center of Pediatric Hematology, Oncology and Immunology named after Dmitriy Rogachev, Ministry of Health of Russia, MoscowRussian Research Institute of Hematology and Transfusiology, Russian Federal Biomedical Agency, Saint PetersburgHematology Research Center, Ministry of Health of Russia, MoscowHematology Research Center, Ministry of Health of Russia, Moscow<p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal blood disorder caused by somatic mutation of the phosphatidylinositol<br />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.</p>http://oncohematology.abvpress.ru/index.php/ongm/article/view/111paroxysmal nocturnal hemoglobinuriaaplastic anemiacomplementhemolysisflow cytometrythrombosisbone marrow transplantationeculizumab |
| spellingShingle | A. D. Kulagin I. A. Lisukov V. V. Ptushkin Ye. R. Shilova N. V. Tsvetaeva Ye. A. Mikhailova National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria Онкогематология paroxysmal nocturnal hemoglobinuria aplastic anemia complement hemolysis flow cytometry thrombosis bone marrow transplantation eculizumab |
| title | National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| title_full | National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| title_fullStr | National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| title_full_unstemmed | National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| title_short | National clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| title_sort | national clinical guidelines for the diagnosis and treatment of paroxysmal nocturnal hemoglobinuria |
| topic | paroxysmal nocturnal hemoglobinuria aplastic anemia complement hemolysis flow cytometry thrombosis bone marrow transplantation eculizumab |
| url | http://oncohematology.abvpress.ru/index.php/ongm/article/view/111 |
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