REPORT OF A FAMILY WITH PELGER-HUET ANOMALY

A 12-year-old boy was seen for abdominal pain and sent to the lab. For CBC. Even though CBC was normal in peripheral smear many of neutrophils (PMN) had only two lobes, with diagnosis of Pelger-Huet anomaly. CBC was performed on parents and three siblings which revealed the same type of anomaly in the mother and the other three children. The father was normal.
This anomaly was first described by Pelger who thought it was due to tuberculosis, later Huet recognized this phenomenon to be inherited as autosomal dominant. Basic anomaly here is limitation of lobing in PMN. In heterozygotes PMN are unsegmented and dumbel - shaped or bilobed and in homozygots the majority of PMN have round nuclei. Incidence is one in 6000 individuals. Except minimal impairment in migration, PMN function is normal.
Acquired Pelger-Huet-Iike change somtimes is seen in chronic infection of bowels, leukemia and non-Hodgkin lymphoma, some drug toxicity such as colchicine, and mycoplama pneumonia infection.