immunology allergic disorders
Chédiak Higashi Syndrome
Treatment of Chédiak Higashi Syndrome
Supportive care using antibiotics, interferon gamma, and sometimes corticosteroids Hematopoietic stem cell transplantation Prophylactic antibiotics can help prevent infections, and interferon gamma can help restore some immune system function. Pulse doses of corticosteroids and splenectomy sometimes induce transient remission of Chédiak-Higashi syndrome. However, unless Hematopoietic Stem Cell Transplantation is done, most patients with Chédiak-Higashi syndrome die of infections by age 7 years. Transplantation of unfractionated human leukocyte antigen (HLA)-identical bone marrow after pretransplantation cytoreductive chemotherapy may be curative. Five-year posttransplantation survival rate is about 60%. 