Длительный устойчивый смешанный химеризм у пациента с синдромом Вискотта-Олдрича после аллогенной трансплантации гемопоэтических стволовых клеток

В статье представлено клиническое наблюдение пациента с синдромом Вискотта–Олдрича (СВО), у которого после проведения 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.   

Abstract: The article describes a clinical case of a patient with Wiskott-Aldrich syndrome (WAS), 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.

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