Pelger-Huet abnormality

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Pelger-Huet abnormality
Pelger-Huet abnormality.
(Image courtesy of Melih Aktan M.D.)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Overview

Pelger-Huet abnormality or Pelger-Huët anomaly (PHA) is a benign dominantly inherited defect of terminal neutrophil differentiation secondary to mutations in the lamin B receptor (LBR) gene.

Laboratory findings

Characteristics observed on blood smears include leukocytes with dumbbell-shaped bilobed nuclei; a reduced number of nuclear segments; and coarse clumping of the nuclear chromatin in neutrophils, lymphocytes, and monocytes.

Differential Diagnosis

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