Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management

Overview

Journal Mediterr J Hematol Infect Dis

Specialty Infectious Diseases

Date 2022 Jan 24

PMID 35070215

Authors

Ilaria Lazzareschi

Elena Rossi

Antonietta Curatola

Giovanna Capozio

Luca Benacquista

Ludovica Iezzi

Donato Rigante

Affiliations

Soon will be listed here.

Abstract

A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections beginning in the first months of life. Of interest is "cyclic neutropenia," an ultra-rare disorder revealed by sinusoidal variations in the neutrophil count and recurring infections every 21 days. Diagnosis of these disorders is frequently obscured by the multiple causes of recurrent fevers in children. The aim of this overview is to outline the physical assessment of children presenting with early-onset symptomatic neutropenia, identify the disease between the many medical conditions and even emergencies which should enter in differential diagnosis, hint at the potential management with granulocyte-colony stimulating factor, define the risk of evolution to hematologic malignancy, and summarize inter-professional team strategies for improving care coordination and outcomes of patients.

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