Systemic mastocytosis is already a common disease in hematological practice

Systemic mastocytosis is a group of diseases of the hematopoietic tissue associated with clonal proliferation of pathological mast cells and their excessive accumulation in organs and tissues of the body with clinical manifestations due to the release of biologically active substances from mastocytes. Symptoms of frequent allergic reactions to various irritants significantly impair the quality of life of patients, and prolonged proliferation of tumor cells leads to disruption of the organs and systems of the body. Also, systemic mastocytosis can accompany other clonal diseases of the hematopoietic tissue. The aggressiveness of the course of various clinical forms of mastocytosis varies significantly from slightly reduced overall survival compared with the population to a fulminant course leading to death within a few months. A significant achievement in improving the prognosis of patients with systemic mastocytosis has been the development of targeted therapy drugs that significantly reduce the risk of death in aggressive forms of the disease. The article provides the most up-to-date information and recommendations for the diagnosis and treatment of patients with systemic mastocytosis. The clinical case of the primary diagnosis of systemic mastocytosis is presented.