Severe congenital autoimmune neutropenia in preterm monozygotic twins: case series and literature review
AbstractThe presence of high levels of neutrophil associated immunoglobulins (NAIG) in the serum of newborns with neutropenia and their mothers is usually associated with the diagnosis of allo-immune neonatal neutropenia (AINN). We describe a set of otherwise healthy late preterm monozygotic twins who presented with an isolated severe neonatal neutropenia on the first day of life. Flow cytometry for neutrophil antibody screen for both twins detected elevated levels of NAIG with normal serum levels of allo anti-neutrophil antibody (allo-NAB). Maternal serum did not contain either NAIG or allo-NAB. Also, the NAIG immunoglobulin M (IgM) levels were markedly increased in both twins if compared to the increase in the NAIG immunoglobulin G (IgG). Both twins showed very good response to a short course treatment with granulocyte colony stimulating factor (G-CSF), they remained clinically well until 12 months of age. We suggest that this case may be an early presentation of autoimmune neutropenia of infancy. This case study is the earliest report of autoimmune neutropenia of infancy in preterm monozygotic twins.