Pelger-Huet Anomaly

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Pelger-Huet Anomaly

Pelger-Huet anomaly is the inherited form of neutrophilic hyposegmentation. Its transmittance is autosomal dominant and the anomaly is present in about one out of every 6000 people. It is due to a mutation in the lamin β-receptor gene.
When present on a peripheral blood smear, more than 70% of the segmented neutrophils will have bi-lobed or mono-lobed nuclei. A bi-lobed nucleus will have two round segments of nearly equal size, connected by a thin chromatin strand. A mono-lobed nucleus may be peanut shaped, slightly indented, or round; chromatin appears fully mature and parachromatin is evident. Pelger-Huet anomaly in the homozygous state has an increased number of cells with singular round nuclei and decreased numbers of the bilobed forms.
The main function of neutrophils is phagocytosis. Neutrophilic function (phagocytosis) is not affected in either the acquired or the inherited anomaly. Since inherited Pelger Huet anomaly is associated with functionally normal neutrophils, the neutrophils are considered a nonpathological variant.
The image to the right is a peripheral blood smear on a Pelger-Huet patient. Note the hyposegmentation of the neutrophils.