Leukocyte Disorders: Quantitative and Qualitative Disorders of the Neutrophil, Part 2

Abstract

The differential diagnosis for a patient presenting with recurrent infections is formidable, given the complexity of the immune system. The clinical presentation of a patient who has a qualitative neutrophil abnormality may be similar to that of one who has an antibody or complement disorder. In general, evaluation for phagocytic cell disorders (Table) should be initiated among those patients who have at least one of the following clinical features: 1) Two or more systemic bacterial infections; 2) Frequent, serious respiratory infections, such as pneumonia or sinusitis, or frequent bacterial infection, such as cellulitis, draining otitis media, or lymphadenitis; 3) Infections presenting at unusual sites (hepatic or brain abscess); and 4) Infections associated with unusual pathogens (ie, Aspergillus pneumonia, disseminated candidiasis, or infections with Serratia marcescens, Nocardia sp, and Pseudomonas cepacia). Disorders of Neutrophil Function HYPERIMMUNOGLOBULIN E SYNDROME (JOB SYNDROME) Hyperimmunoglobulin E (hyper-IgE) syndrome is an autosomal dominant disorder with incomplete penetrance that is characterized by elevated levels of serum IgE and recurrent staphylococcal abscesses (Table). Neutrophils in these children exhibit impaired chemotaxis, although phagocytic function is normal. Clinically, hyper-IgE syndrome must be distinguished from severe atopic dermatitis. Both disorders are characterized by the development of severe dermatitis in the presence of elevated IgE levels.