NEUTROPENIA IN PEDIATRIC PRACTICE: ETIOLOGY, PATHOGENESIS, CLASSIFICATION, DIAGNOSIS, TREATMENT (LITERATURE REVIEW AND OWN OBSERVATIONS)

Abstract

The article is devoted to the problem of diagnosis and treatment of neutropenic conditions in children. A literary review of the modern understanding of the etiology, pathogenetic variants, classification, course, clinical picture, observation and treatment of neutropenic conditions is made. The severity of neutropenia, including agranulocytosis, has been clarified. The types of neutropenia are discussed: congenital (genetically determined), acquired and chronic benign neutropenia of childhood. The differentiation of the competence of pediatric and hematological services has been made. The concept of pseudoneutropenic syndrome is analyzed. The causes of acquired neutropenia are stated. It is emphasized that acquired neutropenia must be differentiated primarily by isoimmune neutropenia of newborns, neutropenia against the background of myelodysplastic syndrome; infectious-associated (including AIDS); nutritional (folate deficiency, vitamin B12 deficiency, copper deficiency, etc.); caused by radiation; drug-induced; associated with a malignant disease. The article presents its own clinical 4-year observation of a patient with Chronic benign neutropenia of childhood. The characteristics of congenital neutropenia and neutropenic syndromes are given, their laboratory and genetic diagnostics are highlighted. The article presents its own clinical, as well as 4-year-old, observation of a child with primary immunodeficiency – severe congenital neutropenia (D72.0), caused by a pathogenic heterozygous mutation in the ELANE gene and unspecified lymphopenia. Differential diagnosis, treatment, and patient follow-up are discussed. The general principles of treating patients with neutropenia are highlighted.

Keywords

• neutropenia
• children
• acquired
• congenital
• treatment.