Pediatria pre prax 4/2020

Neutropenia in children – diagnostic and treatment method

Neutropenia in childhood is a very common phenomenon, it represents a heterogeneous group of acquired and congenital disorders of hematopoiesis with either destruction of mature neutrophils in the peripheral blood (PB) or aberrant maturation of neutrophilic granulocytes in the bone marrow (BM). The pathophysiological mechanisms of these conditions are the subject of extensive research and are not fully understood. Patients with severe congenital neutropenia are prone to recurrent, often life-threatening infections that occur in the first months of life; children with immune-mediated neutrophil destruction have a benign course and rarely require any treatment. The diagnosis is based on clinical manifestations, neutrophil measures in a peripheral blood, bone marrow examination, genetic and immunological tests. The aim of G- CSF treatment is to keep the ANC above 1.0 x 109/L. It is recommended to monitor hematological parameters regularly (once every 3 months) and, in patients with congenital disorders, BM exam once yearly. For patients who do not respond to therapy who proceed to malignant transformation, the only curative treatment is hematopoietic stem cell transplantation.

Keywords: neutropenia, congenital neutropenia, autoimmune neutropenia, cytopenia, myelodysplastic syndrome, hematopoietic stem cell transplantation, growth factor, G-CSF