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.

Significant expansion of telemedicine technologies was made possible by the adoption of the necessary legal regulation and initiation of the national program “Healthcare”. National research centers were assigned a mission to provide advisory and methodological support to the regional hospitals. The manuscript describes the experience of the National Research Center for Hematology in application of telemedicine technologies in order to improve the quality of specialized medical care. Progressive increase in the number of requests for telemedicine consultations was observed during the last 5 years, also due to the activities aimed at expansion of geographical coverage of telemedicine technologies and involvement of the regional doctors. In 2019 1380 requests were received from 80 regions of the Russian Federation. The largest number of requests came from the hospitals of the Central (28%) and Siberian (25%) Federal Districts. Distribution of consultations by aim, disease, regions of origin is presented in the manuscript. Telemedicine consultations significantly contribute to the implementation of precise diagnostics and monitoring of patients with blood disorders, shortening of the time of diagnosis, timely treatment initiation, help to organize correct patient referrals, ultimately reducing the risks of treatment failure, complications and lethal outcomes. Analysis of the data accumulated in the consulting National research center allows to assess the quality and effectiveness of medical care in the regional hospitals.

Background. Since 2018 a widespread national project “Healthcare” has been implemented in the Russian Federation (RF) to improve the quality, efficiency, availability, and affordability of medical care in the profiles of specialties in constituent entities of the RF. Modern hematology as a medical field of high technology and crucial solutions is notable for its multi- and interdisciplinarity of most nosological forms, complexity of diagnostic process, multi-structuredness and diversity of related physician teams in different structural units and subdivisions. One of the key issues in federal proj ects is to determine the indicators for assessing the efficiency of regional hematological services in constituent entities of the RF. Aim. To elaborate and substantiate a new integrated operational efficiency index for hematological services in constituent entities of the RF. Materials & Methods. The analysis of data and assessment of feasibility of a new integrated operational index “early mortality in acute leukemia” (AL) were based on the results of 5 multi-center trials, including an epidemiological one. Results. Multi-center clinical studies on AL are the only objective tools for assessing the treatment efficacy, its improvement, and further training of hematologists taking part in the trials. AL treatment requires well-developed infrastructure of hematological services involving not only staff matters and organization of hematologists’ activities, but also management of many highly important related subdivisions and laboratories, logistics of their interaction, time specifications, meeting clinical guidelines, and lastly, and most importantly, financial support. Conclusion. The Unified State Information System “Hematology” is the only platform providing the objective information on patients’ vital status and enabling the use of the suggested integrated index for assessing the quality and efficiency of hematological services in the regions of the RF. This indicator of early mortality in AL patients less than 60 years of age is 15 % for acute myeloid leukemias and 10 % for acute lymphoblastic leukemias. Its low values would demonstrate that this or that constituent entity of the RF is provided with sufficient infrastructure, technologies, and a professional team to keep those patients alive who have severe but curable hematological diseases. The indicator of long-term survival or “life years gained” should become the main strategic criterion for the therapy efficacy in hematological diseases.