immune disorders
Chédiak Higashi Syndrome
Treatment of Chédiak Higashi Syndrome
Antibiotics to prevent infections Interferon gamma and sometimes corticosteroids Stem cell transplantation Treatment of Chédiak-Higashi syndrome involves antibiotics to help prevent infections and interferon gamma (a drug that modifies the immune system) to help the immune system function better. Corticosteroids and removal of the spleen (splenectomy) sometimes temporarily relieve symptoms. However, unless Stem Cell Transplantation is done, most people die of infections by the time they are 7 years old. Transplantation of stem cells from a donor with the same tissue type may cure them. Usually, children are given a chemotherapy drug before transplantation (pretransplant conditioning chemotherapy) to reduce the risk of rejection of the transplanted cells. About 60% of children are alive 5 years after transplantation. 