Resolution of the Expert council “Unsolved problems of targeted therapy for paroxysmal nocturnal hemoglobinuria in Russia”

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  • Authors: Kulagin A.D.1, Lukina E.A.2, Ptushkin V.V.3,4, Kostinov M.P.5, Arshanskaya E.G.6, Babaeva T.N.7, Ksenzova T.I.8, Fidarova Z.T.2, Marchenko M.V.1
  • Affiliations:
    1. R. M. Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantology of the First St. Petersburg State Medical University named after Academician I. P. Pavlova" Ministry of Health of Russia
    2. Federal State Budgetary Institution "National Medical Research Center of Hematology" of the Ministry of Health of the Russian Federation
    3. Department of Health of Moscow
    4. GBUZ of Moscow "City Clinical Hospital named after S. P. Botkin of the Department of Health of Moscow"
    5. I. I. Mechnikov Scientific Research Institute of Vaccines and Serums
    6. Moscow City Hematology Center of the State Medical Institution of Moscow "S. P. Botkin City Clinical Hospital of the Moscow Department of Health"
    7. Novosibirsk State Medical University of the Ministry of Health of the Russian Federation
    8. GBUZ TO "Regional Clinical Hospital No. 1"
  • Issue: Vol 18, No 3 (2023)
  • Pages: 140-144
  • Section: RESOLUTION
  • Published: 13.09.2023
  • URL: https://oncohematology.abvpress.ru/ongm/article/view/846
  • DOI: https://doi.org/10.17650/1818-8346-2023-18-3-140-144
  • ID: 846

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Abstract

   On June 24, 2023, an Expert Council was held in St. Petersburg, during which leading experts in the field of hematology discussed current achievements and answered a number of unresolved issues of targeted therapy of paroxysmal nocturnal hemoglobinuria (APG) in order to further improve treatment results in Russia. During the Expert Council, the following aspects of targeted APG therapy were considered: • criteria for the suboptimal response of patients with APG to therapy with inhibitors of the 5th component of complement (C5); • efficacy and safety of the use of pegcetacoplan in APG in patients with insufficient efficacy of inhibitors of the C5 component of complement; • vaccination issues before starting therapy with complement inhibitors and the possibility of conducting treatment with pegcetacoplan at home.

About the authors

A. D. Kulagin

R. M. Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantology of the First St. Petersburg State Medical University named after Academician I. P. Pavlova" Ministry of Health of Russia

Author for correspondence.

Alexander Dmitrievich Kulagin, MD, Director, Head of the Department

Department of Hematology, Transfusiology and Transplantology with the course of Pediatric Oncology of the FPO named after prof. B. V. Afanasyev

Saint-Petersburg

Russian Federation

E. A. Lukina

Federal State Budgetary Institution "National Medical Research Center of Hematology" of the Ministry of Health of the Russian Federation

Elena Alekseevna Lukina, MD, Professor, Head of the Department

department of orphan diseases

Moscow

Russian Federation

V. V. Ptushkin

Department of Health of Moscow; GBUZ of Moscow "City Clinical Hospital named after S. P. Botkin of the Department of Health of Moscow"

Vadim Vadimovich Ptushkin, MD, Professor, Chief Freelance Specialist Hematologist, Deputy Chief Physician for Hematology

Moscow

Russian Federation

M. P. Kostinov

I. I. Mechnikov Scientific Research Institute of Vaccines and Serums

Mikhail Petrovich Kostinov, Corresponding Member of the Russian Academy of Sciences, MD, Professor, Head of the Laboratory

laboratory of vaccination and immunotherapy of allergic diseases

Moscow

Russian Federation

E. G. Arshanskaya

Moscow City Hematology Center of the State Medical Institution of Moscow "S. P. Botkin City Clinical Hospital of the Moscow Department of Health"

Evgenia Grigorievna Arshanskaya, PhD, hematologist

Moscow

Russian Federation

T. N. Babaeva

Novosibirsk State Medical University of the Ministry of Health of the Russian Federation

Tatiana Nikolaevna Babaeva, PhD, hematologist, assistant

Faculty of advanced training and professional retraining of doctors

Department of Therapy, Hematology and Transfusiology

Novosibirsk

Russian Federation

T. I. Ksenzova

GBUZ TO "Regional Clinical Hospital No. 1"

Tatiana Ilyinichna Ksenzova, PhD, Head of the Department

Department of Hematology and Chemotherapy

Tyumen

Russian Federation

Z. T. Fidarova

Federal State Budgetary Institution "National Medical Research Center of Hematology" of the Ministry of Health of the Russian Federation

Zalina Taimurazovna Fidarova, PhD, Hematologist, Head of the department

hemoblastosis and hematopoietic depression chemotherapy department with bone marrow and hematopoietic stem cell transplantation unit

Moscow

Russian Federation

M. V. Marchenko

R. M. Gorbacheva Research Institute of Pediatric Oncology, Hematology and Transplantology of the First St. Petersburg State Medical University named after Academician I. P. Pavlova" Ministry of Health of Russia

Maria Viktorovna Marchenko, hematologist

Saint-Petersburg

Russian Federation

References

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  7. Kulagin A., Klimova O., Rudakova T. et al. Benefits and limitations of long-term eculizumab treatment for paroxysmal nocturnal hemoglobinuria (PNH): real-world data from large cohort study in Russia. Blood 2018;132(suppl 1):2589. doi: 10.1182/blood-2018-99-120139
  8. Instructions for use of eculizumab and ravulizumab. State Register of Medicinal Products. Available at: rosminzdrav.ru. (In Russ.).
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  11. www.gov.uk [Internet]. Public Health England. Immunisation of individuals with underlying medical conditions: the green book, chapter 7 [updated Jan 2020]. Available at: The Green book of immunisation – chapter 7 – Immunisation of immunocompromised individuals (https://pulsedive.com/ioc/publishing.service.gov.uk).
  12. Meningococcal vaccines: WHO position paper, November 2011. Wkly Epidemiol Rec 2011;86(47):521–40.
  13. Briko N.I., Namazova-Baranova L.S., Koroleva I.S. et al. Resolution of the Interdisciplinary Expert Council for prevention of severe infections in patients with genetic disorders of complement system regulation receiving eculizumab therapy. Epidemiologiya i vaktsinoprofilaktika = Epidemiology and Vaccinal Prevention 2017;16(1):51–4. (In Russ.).

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