Mastocytosis

Mastocytosis is a rare blood disease (less than 20 cases per 1 million population per year). In rare cases, their formation is caused by the expansion of heterogeneous white line cells, or plasma, called mast cells. They contain substances - heparin and heparan sulfate - that impede the formation of blood clots, which is associated with their protective function (for example, in case of injury) and explains the danger of this disease for the patient’s life. Mastocytosis is divided into primary and secondary forms. Secondary mastocytosis (Marek's disease) may be associated with one of the following blood diseases: * myeloid leukemia; * aplastic anemia; history of uterine bleeding or hemorrhage of various etiologies (hemorrhagic diathesis). The most common cause of the development of the disease is considered to be ionizing radiation. Males are more often susceptible to pathologies. Immunopathogenesis Inflammation caused by infection leads to an increase in the number of stem cells involved in the production of granulocytes. This becomes the basis for the activation of the cell development cycle, which results in the duplication of nuclei and the formation of granules in the cytoplasm within a few days. The products of exchange between nuclei, through interaction, lead to an avalanche-like chain of biochemical changes, provoking the proliferation of the cytoplasm and the division of normal blood cells. Clinical manifestations Infections of various types behave covertly in the lymphoid tissue or bone marrow for a long time, acquiring an asymptomatic or erased clinical picture. This pathology can be painless and discovered by chance during an examination due to another disease or occur under the guise of chronic infections and other pathologies of the gastrointestinal tract or cardiovascular system. The diagnosis is made only with the constant growth of plasma cells and granulocytes in the bone marrow cavities. After this, connective tissue cells gradually grow in the skin, and foci of inflammatory processes begin to form in the liver area. Therefore, the course of the disease is accompanied by a number of symptoms: the gums and scalp become dark brown and begin to actively grow; The general condition of the body also worsens: weakness appears