Clinical guidelines for the management of patients with paroxysmal nocturnal hemoglobinuria-Reference-Cited by-同舟云学术

Clinical guidelines for the management of patients with paroxysmal nocturnal hemoglobinuria

Published:2022-10-23 Issue:3 Volume:67 Page:426-439
ISSN:2411-3042
Container-title:Russian journal of hematology and transfusiology
language:
Short-container-title:Gematologiâ i transfuziologiâ

Author:

Savchenko V. G.¹^ORCID,Lukina E. A.¹^ORCID,Mikhaylova E. A.¹^ORCID,Tsvetaeva N. V.¹^ORCID,Latyshev V. D.¹^ORCID,Lukina K. A.¹^ORCID,Fidarova Z. T.¹^ORCID,Galtseva I. V.¹^ORCID,Dvirnik V. N.¹^ORCID,Ptushkin V. V.²^ORCID,Afanasyev B. V.³^ORCID,Kulagin A. D.³^ORCID,Shilova E. R.⁴^ORCID,Maschan A. A.⁵^ORCID,Smetanina N. S.⁵,Lugovskaya S. A.⁶^ORCID

Affiliation:

1. National Medical Research Center for Hematology

2. Botkin City Clinical Hospital of the Moscow Health Department

3. Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation, Pavlov First State Medical University of St. Petersburg

4. Russian Research Institute of Hematology and Transfusiology of the Federal Medical and Biological Agency

5. Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology

6. Russian Medical Academy of Continuing Professional Education

Abstract

Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disease of the blood system characterized by intravascular hemolysis, bone marrow dysfunction and an increased risk of thrombotic and organ complications.Aim — to provide relevant clinical recommendations for the provision of medical care to adults and children with PNH.Basic information. Experts from the National Hematological Society association which is focused on the promotion of hematology, transfusiology and bone marrow transplantation along with experts from the public organization, National Society of Pediatric Hematologists and Oncologists, have developed current clinical recommendations for providing medical care to adults and children with PNH. The recommendations address in detail the issues of etiology, pathogenesis, epidemiology, and clinical manifestations of the disease. Special attention is paid to the diagnosis, differential diagnosis, and treatment of PNH based on the principles of evidence.

Publisher

National Medical Research Center of Hematology of the Ministry of Health of the Russian Federation

Subject

Hematology

Reference51 articles.

1. Bessler M., Mason P.J., Hillmen P., et al. Paroxysmal nocturnal haemoglobinuria (PNH) is caused by somatic mutations in the PIG-A gene. EMBO J. 1994; 13(1): 110–7. DOI: 10.1002/j.1460-2075.1994.tb06240.x.

2. Takeda J., Miyata T., Kawagoe K., et al. Deﬁciency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria. Cell. 1993; 73(4): 703–11. DOI: 10.1016/0092-8674(93)90250-t.

3. Medof B.Y.M.E., Kinoshita T., Nussenzweig V. Inhibition of complement activation on the surface of cells after incorporation of decay-accelerating factor (DAF) into their membranes. J Exp Med. 1984; 160(5): 1558–78. DOI: 10.1084/ jem.160.5.1558.

4. Lublin D.M., Atkinson J.P. Decay-accelerating factor: Biochemistry, molecular biology, and function. Annu Rev Immunol. 1989; 7: 35–58. DOI: 10.1146/annurev.iy.07.040189.000343.

5. Rollins S.A., Sims P.J. The complement-inhibitory activity of CD59 resides in its capacity to block incorporation of C9 into membrane C5b-9. J Immunol. 1990; 144(9): 3478–83.

Cited by 3 articles. 订阅此论文施引文献订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献

1. Hematological response in patients with paroxysmal nocturnal hemoglobinuria treated with C5-inhibitor;Oncohematology;2024-01-27

2. Diagnostic errors and treatment of anemia caused by vitamin B<sub>12</sub> deficiency;Oncohematology;2024-01-27

3. Resolution of the Expert council “Unsolved problems of targeted therapy for paroxysmal nocturnal hemoglobinuria in Russia”;Oncohematology;2023-09-13