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.

Lazzareschi, I., Rossi, E., Curatola, A., Capozio, G., Benacquista, L., Iezzi, L., Rigante, D., Assessment of congenital neutropenia in children: common clinical sceneries and clues for management, <<MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES>>, 2022; 2022 (14(1)): 1-11. [doi:10.4084/MJHID.2022.008] [http://hdl.handle.net/10807/191863]

Assessment of congenital neutropenia in children: common clinical sceneries and clues for management

Lazzareschi, Ilaria;Rossi, Elena;Benacquista, Luca;Iezzi, Ludovica;Rigante, Donato
2021

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.
2021
Inglese
Lazzareschi, I., Rossi, E., Curatola, A., Capozio, G., Benacquista, L., Iezzi, L., Rigante, D., Assessment of congenital neutropenia in children: common clinical sceneries and clues for management, <<MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES>>, 2022; 2022 (14(1)): 1-11. [doi:10.4084/MJHID.2022.008] [http://hdl.handle.net/10807/191863]
File in questo prodotto:
File Dimensione Formato  
Lazzareschi et al x MJHID.pdf

accesso aperto

Tipologia file ?: Versione Editoriale (PDF)
Licenza: Creative commons
Dimensione 412.57 kB
Formato Adobe PDF
412.57 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/191863
Citazioni
  • ???jsp.display-item.citation.pmc??? 4
  • Scopus 8
  • ???jsp.display-item.citation.isi??? 7
social impact