Named after Karel Pelger (1885-1931), a Dutch specialist in tuberculosis, and Gauthier Huet (1879-1970), a Dutch pediatrician. In 1928, Pelger published an account of a patient with Tb who had neutrophils with bilobed nuclei (a marker of immaturity) and coarse clumped chromatin (a marker of maturity). The patient died, and so the findings were believed to predict a poor prognosis. In 1931, Huet reported similar looking neutrophils in a patient with Tb. Only this time, the cell morphology persisted when the patient recovered. Huet studied her family and found similar forms in healthy relatives, indicating a benign inherited condition. Schilling termed the condition Pelger-Huet anomaly (PHA). Today, two types are recognized: 1) congenital PHA (caused by mutation in the LBR gene), and 2) acquired PHA (aka pseudo PHA), found most commonly in MDS. Neutrophil function is normal in both types.
