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Difference between revisions of "Pelger-Huët anomaly"

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'''Appearance:'''
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Neutrophils with markedly reduced nuclear segmentation – typically just two lobes described as resembling a pair of reading glasses though sometimes a single lobe; the nuclear chromatin pattern is more coarse than usual for neutrophils.
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[[File:Ph1.jpg|leftt|250px|link=]]
 
[[File:Ph1.jpg|leftt|250px|link=]]
 
[[File:Ph2.jpg|leftt|250px|link=]]
 
[[File:Ph2.jpg|leftt|250px|link=]]
 
[[File:Ph3.jpg|leftt|250px|link=]]
 
[[File:Ph3.jpg|leftt|250px|link=]]
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'''''Images of the Pelger-Huët  anomaly:''' The most typical form is the spectacle appearance (image 1). Two further images show mono-lobar forms. A monolobed neutrophil (image 2) or eosinophil (image 3). Mononuclear forms are more frequent in homozygous individuals. The characteristic dense and homogenous nuclear chromatin pattern of the cells is also demonstrated.''
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'''Cause:'''
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Now recognised as a mutation of the lamin B receptor gene. A co-dominant inheritance pattern causes more marked neutrophil anomalies in homozygote individuals (who may also have skeletal anomalies such as polydactyly, short metacarpals, short upper limbs, short stature, or hyperkyphosis).
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'''Described by:'''
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The appearance was first reported in Holland in 1928 by Karel Pelger, then as an inherited disorder in 1931 by Gauthier Jean Huët 
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'''Consider:'''
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In true Pelger-Huët  cases the proportion of abnormal cells is very high and other cells are normal in appearance, be aware of:
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*Pseudo-Pelger-Huët cells arising in haematological disease or in relation to some medication (see separate section)
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*Do not confuse with left-shifted band neutrophils (look for associated activation)
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Revision as of 20:43, 8 November 2019


Appearance:

Neutrophils with markedly reduced nuclear segmentation – typically just two lobes described as resembling a pair of reading glasses though sometimes a single lobe; the nuclear chromatin pattern is more coarse than usual for neutrophils.



leftt leftt leftt

Images of the Pelger-Huët anomaly: The most typical form is the spectacle appearance (image 1). Two further images show mono-lobar forms. A monolobed neutrophil (image 2) or eosinophil (image 3). Mononuclear forms are more frequent in homozygous individuals. The characteristic dense and homogenous nuclear chromatin pattern of the cells is also demonstrated.



Cause:


Now recognised as a mutation of the lamin B receptor gene. A co-dominant inheritance pattern causes more marked neutrophil anomalies in homozygote individuals (who may also have skeletal anomalies such as polydactyly, short metacarpals, short upper limbs, short stature, or hyperkyphosis).


Described by:


The appearance was first reported in Holland in 1928 by Karel Pelger, then as an inherited disorder in 1931 by Gauthier Jean Huët

Consider:

In true Pelger-Huët cases the proportion of abnormal cells is very high and other cells are normal in appearance, be aware of:

  • Pseudo-Pelger-Huët cells arising in haematological disease or in relation to some medication (see separate section)
  • Do not confuse with left-shifted band neutrophils (look for associated activation)