CLINICAL AND ULTRASTRUCTURAL OBSERVATIONS OF BENIGN NEUTROPENIA OF CHILDHOOD AND NEUTROPENIA ASSOCIATED WITH HYPOGAMMAGLOBULINEMIA

Abstract

Chronic neutropenia of childhood and neutropenia associated with hypogammaglobulinemia may have autoimmune etiologies. We observed 9 patients with isolated neutropenia and 5 boys with neutropenia associated with hypogammaglobulinemia. Both types of patients presented between 4 and 24 months of age with recurrent otitis media, skin infections caused by S. aureus, P. aeruginosa or Candida and chronic diarrhea. Only the 5 patients with hypogammaglobulinemia developed invasive infections such as bacterial pneumonias and sepsis due to pseudomonas and clostridia. No patients developed an invasive infection while receiving gammaglobulin. No demargination was observed after epinephrine, and no sustained response occurred with steroid therapy. Anti-neutrophil antibodies were not detected in 10 of 12 patients. All patients with hypogammaglobulinemia had elevated percents of T4 lymphocytes. Marrow aspirates showed deficiency of granulocytes beyond the myelocyte or metamyelocyte stage. Ultrastructural studies showed reduction in secondary granules and increased lucency of primary granules. However, myeloperoxidase content was normal by electronmicroscopy. This morphologic appearance may represent accelerated neutrophil senescence. Analysis of both the clinical course and laboratory findings in patients with isolated neutropenia, or with neutropenia associated with hypogammaglobulinemia, thus revealed no distinguishing characteristics. Neutropenia may have a common pathogenesis in both groups of patients.