抄録
Clinical observations were performed in 7 patients with defective neutrophil chemotaxis and hyper-IgE, with special emphasis on the hematologic and immunologic findings such as neutrophil and monocyte chemotaxis. The patients ranged in age from 7(2/12) to 23 years old, and 5 were male and 2 female. In vitro neutrophil chemotaxis was studied by the agarose plate method and by a modification of the Boyden's method. In vitro monocyte chemotaxis was examined by the agarose plate method. The patients had suffered from chronic severe dermatitis and recurrent pyogenic infections since infancy. Major infections included pneumonia, bronchitis, superficial abscess, lymphadenitis and otitis media. The frequency and severity of their infections varied widely from patient to patient. Neutrophil chemotaxis was impaired in all of the patients. In some patients, however, there was a tendency for neutrophil chemotaxis to improve during the observation period. Monocyte chemotaxis was defective in two of the 7 patients. Eosinophilia was observed in most of them. Serum IgG levels were normal to elevated, and IgA and IgM were normal. Serum IgE levels were elevated, ranging from 677 to 11,000 IU/ml. Lymphocyte subpopulation was normal. In our patients, chronic dermatitis and hyper-IgE were persistent abnormalities, but there was no apparent relationship between any pairs of dermatitis, neutrophil chemotaxis defect, and serum IgE level. Our observations indicate that this disorder is heterogeneous not only in the clinical features but also in the hematologic and immunologic findings.
