Approach to the patient with neutropenia in childhood

Abstract

Neutrophils have an important role in host defense and acute inflammation. It is well known that susceptibility to infection increases when the neutrophil count is low. Neutropenia were classified as mild, moderate and severe according to the neutrophil counts, or acute and chronic depending on the duration of neutropenia, or congenital and acquired according to the mechanism. The patients with neutropenia are clinically different due to underlying mechanism, they have life-threatening infections or no infection may be observed. The most common cause of acquired neutropenia is viral infection, followed by drugs and autoimmune neutropenia. Congenital neutropenia are usually diagnosed by acute and life-threatening invasive bacterial and fungal infections. Immune system disorders and other systemic abnormalities may be accompanied or not. Recent years, novel single gen defects causing congenital neutropenia were defined through advanced genetic techniques. Molecular diagnosis is useful for risk stratification, choice of therapy and prognosis on follow-up. This review was prepared for pediatricians as a guide focused on approach neutropenia, which tests should be performed and when should be referred to a specialist. (Turk Pediatri Ars 2015; 50: 136-44)

Autoimmune Transient Neutropenia: A Case Report

“…In general, the disease has good prognosis and neutropenia improves spontaneously. Although the total neutrophil count varied from 0 to 150/mm 3 , infections are mild [2].…”

“…Congenital neutropenia is usually diagnosed by invasive acute bacterial or fungal infections, which are life-threatening [2].…”

“…Persistent Neutropenia requires investigation as it entails infectious complications. Nowadays, cytogenetic studies on bone marrow are considered crucial for isolated neutropenia investigation, especially when it is suspected to be congenital [4]: the major determinant in the relationship between neutropenia and infection is the neutrophil count in bone marrow [2].…”

“…Patients who use this drug have normalized neutrophil count, reduced recurrent infections and inflammatory symptoms reduced by over 90%. Good responses for congenital neutropenia are obtained with high doses of G-CSF [2].…”

“…Primary autoimmune neutropenia is classified as an acquired neutropenia and is a rare disease. It is more commonly observed in girls and children younger than two years old [2]. In most cases, the course of the disease is benign and self-limited [6].…”