Pediatric Hematology/Oncology and ImmunopathologyPediatric Hematology/Oncology and ImmunopathologyВопросы гематологии/онкологии и иммунопатологии в педиатрии1726-17082414-9314Fund Doctors, Innovations, Science for Children51810.24287/1726-1708-2021-20-2-148-155SCHOOL OF IMMUNOLOGY – EXPERT OPINIONШКОЛА ИММУНОЛОГА – МНЕНИЕ ЭКСПЕРТАLong-term persistent mixed chimerism in a patient with Wiskott–Aldrich syndrome after allogeneic hematopoietic stem cell transplantationДлительный устойчивый смешанный химеризм у пациента с синдромом Вискотта–Олдрича после аллогенной трансплантации гемопоэтических стволовых клетокBludovaV. О.БлудоваВ. О.
Russian Federation

Viktoriya O. Bludova, a resident 

 1 Samory Mashela St., Moscow 117997

Блудова Виктория Олеговна, врач-ординатор 

 117997, Москва, ул. Саморы Машела, 1

voevodina_1996@list.ruLaberkoA. L.ЛаберкоА. Л.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

RodinaYu. A.РодинаЮ. А.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

BrilliantovaV. V.БриллиантоваВ. В.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

RaykinaE. V.РайкинаЕ. В.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

KhorevaA. L.ХореваА. Л.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

PershinD. E.ПершинД. Е.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

TereshchenkoG. V.ТерещенкоГ. В.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

ShcherbinaA. Yu.ЩербинаА. Ю.
Russian Federation

 1 Samory Mashela St., Moscow 117997

117997, Москва, ул. Саморы Машела, 1

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of the Russian FederationФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава России220520212021481552205202122052021Copyright ©; 2021, «D. Rogachev NMRCPHOI»Copyright ©; 2021, ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава России2021«D. Rogachev NMRCPHOI»ФГБУ «НМИЦ ДГОИ им. Дмитрия Рогачева» Минздрава Россииhttps://creativecommons.org/licenses/by/4.0https://hemoncim.com/jour/article/view/518

The article describes a clinical case of a patient with Wiskott–Aldrich syndrome, in whom long- term persistence of mixed chimerism was determined after hematopoietic stem cell transplantation (HSCT) from a haploidentical donor. Based on the analysis of the patient's clinical picture after HSCT, it was shown that the presence of> 50% of donor cells in the myeloid lineage is necessary for the correction of thrombocytopenia. In addition, the presence of mixed chimerism in B-lymphocytes possibly contributed to the development of autoimmune complications in the patient, as well as to the persistent hypogammaglobulinemia, despite the restoration of the normal numbers of lymphocytes in all main sub-populations. The role of mixed chimerism in the pathogenesis of immune post-transplant complications requires study in large groups of patients with primary immunodeficiencies. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

В статье представлено клиническое наблюдение пациента с синдромом Вискотта–Олдрича, у которого после трансплантации гемопоэтических стволовых клеток (ТГСК) от гаплоидентичного донора определялась длительная персистенция смешанного химеризма. На основании анализа клинической картины пациента после ТГСК было показано, что для коррекции тромбоцитопении необходимо присутствие > 50% донорских клеток в миелоидной линии. Кроме того, наличие смешанного химеризма в В-лимфоцитах внесло свой вклад в развитие аутоиммунных осложнений у пациента, а также к стойкой гипогаммаглобулинемии, несмотря на восстановление нормального числа лимфоцитов всех популяций. Роль смешанного химеризма в патогенезе иммунных посттрансплантационных осложнений требует изучения на больших группах пациентов с первичными иммунодефицитами. Родители пациента дали согласие на использование информации, в том числе фотографий ребенка, в научных исследованиях и публикациях.

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