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Pelger-Huët anomaly

From www.haematologyetc.co.uk


Appearance:

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



Images of the Pelger-Huët anomaly

The most typical form is the spectacle appearance seen in heterozygotes (image 1). Two further images show monolobed forms. A monolobed neutrophil (image 2) or eosinophil (image 3). Monolobed 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 homozygous 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 cases of Pelger-Huët anomaly, the proportion of abnormal cells is very high; while other blood cells are normal in appearance. However, be aware of:

  • Pseudo-Pelger-Huët cells arising as part of haematological diseases (such as MDS) or in relation to particular medication (see pseudo Pelger cells)
  • Do not confuse them with left-shifted band neutrophils (look for associated activation)