Recurrent attacks of abdominal pain and fever with familial segmentation arrest of granulocytes.

INTRODUCTION T HE CLINICAL HARMLESSNESS ofthe Pelger-Huet anomaly has been established. The anomaly is characterized by arrested segmentation of granulocyt#{231}s, typical appearance of the granulocytic nuclei, and autosomal dominant inheritance. The affected persons have no clinical complaints. Here we describe a family in which four sisters who have a segmentation arrest of granulocytes resembling the Pelger-Huet anomaly suffer from paroxysmal bouts of abdominal pain and fever. Despite extensive investigations no explanation has been found for the malady.