Leukocyte Adhesion Defect Type I: Delayed Umbilical Cord Separation, a Surplus of Cells, and a Deficit of Defense

A 3-month-old male infant presented with fever and irritability. He had a history of delayed umbilical cord separation (detachment on day 28) and had previously been evaluated for umbilical granuloma and malodorous discharge (Figures 1A, B). At age two months, while abroad, he was hospitalized for umbilical stump bleeding. Labs showed elevated C-reactive protein (CRP, 8.5 mg/dL), erythrocyte sedimentation rate (ESR, 48 mm/hour), and leukocytosis (white blood cells [WBC] 28,500/mcL). At that time, leukemia was excluded via peripheral smear and bone marrow aspirate.