IMMUNOLOGICAL AND BIOCHEMICAL PROFILES IN RESPONSE TO TRANSFUSION THERAPY IN AN ADENOSINE DEAMINASE-DEFICIENT PATIENT WITH SEVERE COMBINED IMMUNODEFICIENCY DISEASE

A child with severe combined immunodeficiency (SCID) and adenosine deaminase (ADA) deficiency has been treated with irradiated frozen red blood cells and irradiated cryoprecipitate-removed fresh frozen plasma. This therapy has been beneficial clinically and the patient has been free of infection for 24 months. Partial immune reconstitution has occurred. The cellular immune response has varied greatly between markedly decreased and near normal function. In spite of a progressive increase of immunoglobulins to normal levels, specific antibody production has been very poor and in vitro immune functions have been variable with low or transiently normal responses. Activities of ADA in red cells, lymphocytes, and granulocytes were low at the time of diagnosis and have remained low in lymphocytes and granulocytes despite transfusion of red cells with normal levels of ADA. © 1979.