Federal clinical guidelines. Primary immunodeficiency: severe combined immunodeficiency

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Abstract

Severe combined immunodeficiency (SCID) is the most life-threatening form of primary immunodeficiency, fatal within the first years of life if hematopoietic stem cell transplantation (HSCT) is not performed. Early diagnosis is crucial for prevention of multiple life-threatening complications, which in turn allows for successful HSCT. Current publication contains clinical recommendations for diagnosis of SCID and its complications, complex treatment, including HSCT, prenatal diagnostics and genetic family counselling.

About the authors

Yulia A. Rodina

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

Author for correspondence.
Email: rodina.julija@rambler.ru
ORCID iD: 0000-0001-9857-4456

allergologist-immunologist of Immunology Department, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), MD, PhD

Russian Federation, Moscow

Elena V. Deripapa

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

Email: rodina.julija@rambler.ru
ORCID iD: 0000-0002-9083-4783

allergologist-immunologist of Immunology Department, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), MD, PhD

Russian Federation, Moscow

Alexandra L. Laberko

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

Email: Alexandra-lab@yandex.ru
ORCID iD: 0000-0002-2354-2588

PhD, MD of immunology department Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID)

Russian Federation, Moscow

Dmitry E. Pershin

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

Email: dimprsh@icloud.com
ORCID iD: 0000-0002-6148-7209

Head of Laboratory of Transplantation Immunology and Immunotherapy of Hemoblastosis, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Russian Federation, Moscow

Ekaterina V. Kalinina

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

Email: rodina.julija@rambler.ru
ORCID iD: 0000-0002-4029-829X

researcher of Laboratory of Molecular Immunology, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Russian Federation, Moscow

Elena V. Raikina

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

Email: e_raikina@inbox.ru
ORCID iD: 0000-0002-7634-2053

head of the Laboratory of Molecular Biology, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation

Russian Federation, Moscow

Anna A. Roppelt

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

Email: roppelt_anna@mail.ru
ORCID iD: 0000-0001-5132-1267

allergologist-immunologist of Immunology Department, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), MD

Russian Federation, Moscow

Darya V. Yukhacheva

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

Email: yudashechka@mail.ru
ORCID iD: 0000-0001-9078-8206

allergologist-immunologist of Immunology Department, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), MD

Russian Federation, Moscow

Vasiliy I. Burlakov

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

Email: sarovas@yandex.ru
ORCID iD: 0000-0003-1267-9957

allergologist-immunologist of Immunology Department, Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), MD

Russian Federation, Moscow

Dmitry N. Balashov

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

Email: Bala8@yandex.ru
ORCID iD: 0000-0003-2689-0569

head of the Department of Hematopoietic Stem Cell Transplantation No. 2 Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency(ESID), PhD

Russian Federation, Moscow

Alexandr G. Rumiantsev

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

Email: alexrum47@mail.ru
ORCID iD: 0000-0002-1643-5960

president of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of Russia, President of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Hematology (ESH), PhD, professor, Academician of the Russian Academy of Science

Russian Federation, Moscow

Anna Yu. Shcherbina

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

Email: shcher26@hotmail.com
ORCID iD: 0000-0002-3113-4939

head of Immunology Department Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of the Russian Federation, member of the National Association of Experts of Primary Immunodeficiency (NAEPID), member of the National Society of Pediatric Hematologists and Oncologists (NSPHO), member of the European Society of Primary Immunodeficiency (ESID), PhD, professor

Russian Federation, Moscow

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Supplementary files

Supplementary Files
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2. Fig. 1. Diagram of the main mechanisms of formation and differentiation of T-lymphocytes [4]

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3. Fig. 2. Diagram representing the frequency of detection of various genetic defects underlying SCID

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4. Fig. 3. Algorithm for the approach to HSCT in SCID

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