A family with congenital Pelger–Huët anomaly

Excerpt

A 30-year-old female was admitted to our hospital with nephrotic syndrome. She did not have any significant previous medical history or abnormal blood examination results. A complete blood cell count (CBC) with differentials using an automated cell counter showed normal results. Since the white blood cell counts had increased after glucocorticoid therapy (prednisolone 1 mg/kg/day) for nephrotic syndrome, the differential white blood cell count was examined under direct visual observation. The peripheral blood smear revealed 63.0% neutrophils with symmetrical bilobed nuclei and normal granulation (Fig. 1a). No immature granulocytes or abnormalities of red blood cells and platelets were observed. She did not have any symptoms indicative of infections or hematological malignancies. The bilobed neutrophils persisted after normalization of white blood cell counts following reduction of glucocorticoid doses. We also screened peripheral blood smears from both of the patient’s parents. Her father’s neutrophils showed a similar abnormality (Fig. 1b). However, her mother’s smears were normal (Fig. 1c). Her father did not have any significant previous medical history or abnormal blood examination results. Both the patient and her father had no obvious musculoskeletal abnormalities. A diagnosis of congenital Pelger–Huët anomaly (PHA) was made based on the familial resemblance of the neutrophils.

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