Immunotherapy of two patients with hyper-immunoglobulin E syndrome with recombinant granulocyte colony stimulating factor and polyvalent gammaglobulin

Hyper-immunoglobulin E (IgE) syndrome is a rare immunodeficiency disease wi th unknown pathophysiology characterized by recurrent infections, eczema an d high serum IgE levels. We report two brothers (aged 29 and 34 years old) with hyper-IgE syndrome who showed marked depression of cellular and humora l immunity including lymphocyte subpopulations and,inmunoglobulin subclasse s (predominantly IgG2) and who frequently suffered from skin and respirator y tract infections. Both were treated with recombinant human granulocyte co lony stimulating factor (rhG-CSF) at a dose of 3-6 mug/kg body weight, thri ce a week for 8 weeks. Since rhG-CSF treatment did not lead to clinical imp rovement, therapy with intravenous gammaglobulin (20 g once a month) follow ed. After a period of 8 months there was only one episode of pneumonia in o ne of the patients while no change was seen in the incidence of other infec tions or in consumption of antibiotics. Thus, this,inmunotherapy was ineffi cient. In conclusion, individual immunological and clinical characteristics of hyper-IgE syndrome may be responsible for the varying efficiency of imm unotherapy.