Inherited Disorder: Pelger-Huët Anomaly

Abstract

Unfamiliarity with the appearance of immature neutrophils and mature neutrophils with bilobed nuclei and coarse clumping of the nuclear chromatin can lead to their erroneous classification. Presence of Pelger-Huët cells might be mistaken for a shift to the left, which represents an increase in the number of un-segmented (band) neutrophils in the circulation. Pelger-Huët anomaly, rather rare disorder is an inherited failure of the nuclei of neutrophils (and also eosinophils) to mature to the normal segmented form. PHA occurs secondary to mutations in the lamin B receptor. Consequently, a left shift always appears to be present. However, distinguishing PHA with acquired or pseudo-Pelger-Huët anomaly (PPHA), which has very similar morphologic characteristics, but it is associated with different pathological states, is very important.