ABSTRACT
There are at least three series, each of which has reported more than 50 children with autoimmune neutropenia, emphasizing how common it is (52-54). These series have all documented the lack of life-threatening infections, which is helpful in considering the diagnosis but confusing in regard to deciding when to treat. Sepsis in these patients is virtually unheard of despite neutrophil counts that are frequently less than 200/,.U. Meningitis may never have been reported in these patients. The series by Bux and Mueller-Eckhardt (53) focused on the incidence of proven pneumonia, but even this is distinctly uncommon, occurring in 10% or less of all infants. On the other hand, perirectal and other superficial, i.e., breast, abscesses in young girls are not rare, and chronic recurrent otitis media is also frequent (55). The latter may result in hearing loss and speech delay. Long-term studies have not been conducted to defme the true incidence of this complication. Similarly, there are no reports of chronic gingivitis and the loss of teeth, but this may also be underreported with lack of follow-up; it is an important problem in Kostman patients.
