Snapshot A 4-day-old girl born to a G1P1 mother with no complications presents with a fever. There is some swelling with serosanguinous fluids around her rectum. Cultures from the fluid grow Staphylococcus spp and is treated with antibiotics. On day 15 of life, she develops an ear infection and is once again treated with antibiotics. The umbilical cord is still attached, and on day 34 of life, the umbilical stump is noted to be erythematous, indurated, and tense. A completel blood cell count is significant for leukocytosis. Immunoglobulin levels are normal. Flow cytometry reveals decreased expression of CD18 to < 1%. Introduction Primary immunodeficiency resulting from phagocytic dysfunction Genetics login to view 2 more bullets Epidemiology login to view 2 more bullets Pathogenesis login to view 7 more bullets Prognosis login to view 4 more bullets Presentation Symptoms login to view 11 more bullets Evaluation ↑ Neutrophils in the absence of infection login to view 1 more bullet Histology login to view 2 more bullets Flow cytometry login to view 1 more bullet Differential Diagnosis Chédiak-Higashi syndrome Chronic granulomatous disease Treatment Hematopoietic stem cell transplant login to view 1 more bullet Prophylactic antibiotics Complications Life-threatening infections Graft versus host disease after stem cell transplant