Beneficial effects of high-dose intravenous gammaglobulin on the accelerated phase of Chediak-Higashi syndrome.
The Chediak-Higashi syndrome (CHS) is a rare, autosomal-recessively inherited disease characterized by giant cytoplasmic granules in neutrophils, monocytes and lymphocytes. It has been reported that a lymphoma-like illness develops in the accelerated phase of the syndrome and that early death ensues. Treatment of CHS has not been established, particularly when the accelerated phase develops. We treated a 15-year-old boy, who had entered into the accelerated phase, with high-dose intravenous gammaglobulin. The treatment was beneficial in the management of accelerated phase of CHS.
D000293:Adolescent; D002609:Chediak-Higashi Syndrome / Q000276:immunology / Q000628:therapy*; D004305:Dose-Response Relationship, Drug; D006801:Humans; D007111:Immunity, Cellular; D007116:Immunization, Passive; D007136:Immunoglobulins / Q000378:metabolism; D008297:Male
D007136:Immunoglobulins