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Related Experiment Video

Updated: Jun 7, 2026

Neutrophil Lifespan Extension with CLON-G and an In Vitro Spontaneous Death Assay
05:52

Neutrophil Lifespan Extension with CLON-G and an In Vitro Spontaneous Death Assay

Published on: May 12, 2023

Cyclic and chronic neutropenia.

David C Dale1, Karl Welte

  • 1Department of Medicine, University of Washington, Seattle, WA 98195, USA. dcdale@u.washington.edu

Cancer Treatment and Research
|November 6, 2010
PubMed
Summary
This summary is machine-generated.

Severe chronic neutropenia, including cyclic and congenital forms, increases infection risk. Both conditions are rare hematological disorders that respond well to granulocyte colony-stimulating factor (G-CSF) treatment.

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The Isolation and Characterization of Low- and Normal- Density Neutrophils from Whole Blood
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The Isolation and Characterization of Low- and Normal- Density Neutrophils from Whole Blood

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Last Updated: Jun 7, 2026

Neutrophil Lifespan Extension with CLON-G and an In Vitro Spontaneous Death Assay
05:52

Neutrophil Lifespan Extension with CLON-G and an In Vitro Spontaneous Death Assay

Published on: May 12, 2023

The Isolation and Characterization of Low- and Normal- Density Neutrophils from Whole Blood
07:17

The Isolation and Characterization of Low- and Normal- Density Neutrophils from Whole Blood

Published on: February 7, 2025

Area of Science:

  • Hematology
  • Genetics
  • Immunology

Background:

  • Severe chronic neutropenia (SCN) is characterized by low neutrophil counts (<0.5 × 10^9/L), leading to heightened susceptibility to life-threatening bacterial infections.
  • This chapter details cyclic neutropenia (CN) and severe congenital neutropenia (SCN), two rare hematological disorders.
  • Both CN and SCN are often linked to mutations in the neutrophil elastase (ELA-2 or ELANE) gene.

Purpose of the Study:

  • To describe the fundamental characteristics of cyclic and congenital neutropenia.
  • To address current clinical challenges in diagnosing and managing these rare hematological conditions.
  • To highlight the efficacy of granulocyte colony-stimulating factor (G-CSF) in treating neutropenia.

Main Methods:

  • Review of existing literature on cyclic and congenital neutropenia.
  • Analysis of genetic mutations associated with these disorders (e.g., ELANE, HAX-1, G6PC3).
  • Discussion of clinical manifestations, diagnostic criteria, and management strategies, including G-CSF therapy.

Main Results:

  • Cyclic neutropenia presents with cyclical drops in neutrophil counts every 21 days, causing symptoms like mouth ulcers and infections.
  • Severe congenital neutropenia involves persistently low neutrophil levels, posing an even greater infection risk.
  • Granulocyte colony-stimulating factor (G-CSF) is an effective treatment for both conditions, though dosage requirements vary in SCN.
  • A subset of SCN patients (10-30%) may develop acute myeloid leukemia, necessitating vigilant monitoring.

Conclusions:

  • Cyclic and congenital neutropenia are rare but serious hematological disorders requiring prompt diagnosis and management.
  • Granulocyte colony-stimulating factor (G-CSF) is a crucial therapeutic agent for improving neutrophil counts and reducing infection risk.
  • Genetic factors play a significant role, and ongoing monitoring is essential, particularly for the potential development of acute myeloid leukemia in SCN patients.