Neutropenia is hereditary

Neutropenia hereditary - a group of rare hereditary diseases with almost complete absence of neutrophils in the blood, detectable permanently (persistent neutropenia) or at regular intervals (periodic neutropenia). As a rule, diseases are inherited recessively. The pathogenesis of the primary mechanisms of neutropenia has been poorly studied, infectious inflammatory processes are caused by neutropenia.

The clinical picture is the propensity to infections (as a rule, staphylococcal), severe alveolar pyorrhea from childhood (naked necks of the teeth, their loosening and prolapse). With the periodic form of the disease - fever and infections are strictly periodic and correspond to days of neutropenia. In the blood, neutrophils are almost completely absent, the number of eosinophils and monocytes is increased. With a periodic form, these changes last several days, appearing through strictly defined intervals of time. Anemia, there is no thrombocytopenia. In the bone marrow there is a rupture of neutrophil maturation at the stage of promialocyte (less often myelocyte), monocytosis (often there are many promonocytes in children), eosinophilia.

Treatment. Antibiotics in the presence of infectious complications, continuous therapy of alveolar pyorrhea.

Forecast. Elimination of infections helps to save the lives of sick children; Over the years, the severity of the disease is weakening.

Prevention. When the form is periodically prescribed antibiotics (for example, oxacillin) before the crisis, which can dramatically weaken the severity of the infection.