CLINICAL GUIDELINES FOR THE MANAGEMENT OF PATIENTS WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

被引：0

作者：

Savchenko, V. G. ^{[1
]}

Lukina, E. A. ^{[2
]}

Mikhaylova, E. A. ^{[3
]}

Tsvetaeva, N., V ^{[2
]}

Latyshev, V. D. ^{[2
]}

Lukina, K. A. ^{[2
]}

Fidarova, Z. T. ^{[4
]}

Galtseva, I., V ^{[5
]}

Dvirnik, V. N. ^{[6
]}

Ptushkin, V. V. ^{[7
]}

Afanasyev, B., V ^{[8
]}

Kulagin, A. D. ^{[8
]}

Shilova, E. R. ^{[9
]}

Maschan, A. A. ^{[10
]}

Smetanina, N. S. ^{[10
]}

Lugovskaya, S. A. ^{[11
]}

机构：

[1] Natl Med Res Ctr Hematol, Moscow 125167, Russia

[2] Natl Med Res Ctr Hematol, Dept Orphan Dis, Moscow 125167, Russia

[3] Natl Med Res Ctr Hematol, Dept Intens High Dose Chemotherapy Hemoblastosis, Moscow 125167, Russia

[4] Natl Med Res Ctr Hematol, Dept Hemoblastosis & Hematopoiet Depress, Moscow 125167, Russia

[5] Natl Med Res Ctr Hematol, Lab Immunophenotyping Blood Cells & Bone Marrow, Moscow 125167, Russia

[6] Natl Med Res Ctr Hematol, Reg Clin & Diagnost Lab, Moscow 125167, Russia

[7] Botkin City Clin Hosp, Moscow Hlth Dept, Hematol, Moscow 125284, Russia

[8] Pavlov First State Med Univ St Petersburg, Raisa Gorbacheva Mem Res Inst Children Oncol Hema, St Petersburg 197022, Russia

[9] Fed Med & Biol Agcy, Russian Res Inst Hematol & Transfusiol, St Petersburg 191024, Russia

[10] Dmitry Rogachev Natl Med Res Ctr Pediat Hematol O, Inst Hematol Immunol & Cell Technol, Moscow 117997, Russia

[11] Russian Med Acad Continuing Profess Educ, Dept Clin Lab Diagnost, Moscow 127994, Russia

来源：

GEMATOLOGIYA I TRANSFUZIOLOGIYA | 2022年 / 67卷 / 03期

关键词：

paroxysmal nocturnal hemoglobinuria; anemia; complement system; flow cytometry; eculizumab; COMPLEMENT INHIBITOR ECULIZUMAB; STEM-CELL TRANSPLANTATION; DECAY-ACCELERATING FACTOR; ICCS/ESCCA CONSENSUS GUIDELINES; GPI-DEFICIENT CELLS; PNH; DIAGNOSIS; ACTIVATION; DISORDERS; TRANSFUSION;

D O I：

10.35754/0234-5730-2022-67-3-426-439

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：426 / 439

页数：14

共 51 条

[1] PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA (PNH) IS CAUSED BY SOMATIC MUTATIONS IN THE PIG-A GENE [J].