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Treatment aims to restore the immune response and prevent infection. Histocompatible bone marrow transplantation is the only satisfactory treatment available to correct immunodeficiency. Because bone marrow cells must be human leukocyte antigen and mixed leukocyte culture matched, the most common donors are histocompatible siblings. However, because bone marrow transplant can produce a potentially fatal graft-versus-host (GVH) reaction, newer methods of bone marrow transplant that eliminate GVH reaction (such as lectin separation and the use of monoclonal antibodies) are being evaluated.
Fetal thymus and liver transplants have achieved limited success. Immune globulin administration may also play a role in treatment. Some SCID infants have received long-term protection by being isolated in a completely sterile environment. However, this approach isn’t effective if the infant already has had recurring infections.
Gene therapy is being used to treat ADA deficiency.
Source: Professional Guide to Diseases (Eighth Edition), 2005
Restoring immune response and preventing infection are the first goals of treatment. Histocompatible bone marrow transplant is the only satisfactory treatment available to correct immunodeficiency.
Because bone marrow cells must be matched according to human leukocyte antigen and mixed leukocyte culture, the most common donors are histocompatible siblings. But bone marrow transplant can produce a potentially fatal graft-versus-host (GVH) reaction, so newer methods of bone marrow transplant that eliminate GVH reaction (such as lectin separation and the use of monoclonal antibodies) are being evaluated.
Fetal thymus and liver transplants have achieved limited success. Administration of immune globulin may also play a role in treatment. Some SCID infants have received long-term protection by being isolated in a completely sterile environment. However, this approach isn’t effective if the infant already has had recurring infections.
Gene therapy is being used for ADA deficiency.
Source: Handbook of Diseases, 2003
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