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A One-Day-Old Girl With Neutropenia

A female neonate, born at 35 weeks gestation via repeat Caesarian section due to premature rupture of membranes, was noted on day 1 of life to have an absolute neutrophil count (ANC) of 0/μL on complete blood count (CBC) obtained after delivery. Hemoglobin was 19.3 g/dL and platelets were 237,000 /uL. The rupture of membranes was not prolonged, with only 6 hours elapsing between the time of rupture and the time of delivery. There were no signs of fetal distress identified on routine monitoring prior to delivery. Other than an initial low blood sugar that persisted through the first 2 days of life, she was well appearing and without dysmorphic features on physical examination. There was a known family history of neutropenia in the patient’s sister (child of the same mother and father as our patient), who is 2 years older. She was found to be neutropenic at birth and required admission to the neonatal intensive care unit (NICU) and granulocyte-colony stimulating factor (G-CSF) injections for 2 months. She did not have any infections during her newborn course. Because of the patient’s neutropenia, she was transferred from the term nursery to the neonatal intensive care unit, where blood cultures were obtained and she underwent a 48-hour period of observation on antibiotics. A repeat CBC on day 2 of life confirmed an ANC of 0/μL. The pediatric hematology/oncology team was consulted to assist with the workup for the neutropenia. Because of the family history of neutropenia, blood work was obtained from the patient’s parents for testing for neonatal alloimmune neutropenia (NAN). After 48 hours of antibiotics, her blood cultures remained negative and the patient remained well appearing without signs of infection. She was started on G-CSF on day 2 of life and was monitored daily. Her neutropenia was initially refractory to G-CSF. However, after titrating the dose up to 10 μg/kg/d, her ANC steadily increased with an ANC of 910/μL by day 10 of life. She was discharged from the NICU on day 11 of life and subsequently was followed closely in pediatric hematology clinic. Her ANC peaked at 38 750/μL on day 22 of life. She was taken off G-CSF that day and her ANC remained stable at greater than 900/μL at each subsequent follow-up visit thereafter. Clinical Course