Autoimmune lymphoproliferative syndrome (Review of literature)

Programmed cell death (apoptosis) of lymphocytes is an integral component of immune homeostasis. Disorders in this process can lead to the development of the autoimmune lymphoproliferative syndrome (ALPS), a unique genetically determined clinical condition in which disorders in lymphocyte apoptosis are combined with lymphoproliferation and autoimmunity. Since the first description of this syndrome in the 20^th century, the potentialities of its diagnosis and treatment have been improved. The hereditary genetic defect involves the FAS signal protein in the majority of ALPS patients, but the notion of unspecified ALPS is gradually blurred with the development of molecular genetic diagnosis - new mutations are identified, extending the spectrum of clinical manifestations and the age of the disease debut. Modern therapeutic methods allow effective control of the disease course.

аутоиммунный лимфопролиферативный синдром, апоптоз, ген FAS, дубль-негативные Т-клетки, критерии диагностики, дифференциальный диагноз, лечение, сиролимус, прогноз, autoimmune lymphoproliferative syndrome, apoptosis, FAS gene, double-negative T-cells, diagnostic criteria, differential diagnosis, treatment, sirolimus, prognosis