Patients with SCID due to ADA deficiency may be eligible for this study. Patients may or may not have received enzyme replacement therapy or gene transfer therapy, or both. The procedure can be done under local anesthesia or light sedation. Injection of small amounts of fluids into the arm to study if the patient's lymphocytes respond normally. Administration of vaccination shots.
Whole blood is collected through a needle placed in an arm vein.
The blood circulates through a machine that separates it into its components. The white cells are then removed, and the red cells, platelets and plasma are returned to the body, either through the same needle used to draw the blood or through a second needle placed in the other arm. Blood drawings to obtain and study the patient's lymphocytes. This study is not currently recruiting new subjects. Overproduction of adenosine deoxynucleosides and deoxynucleotides in adenosine deaminase deficiency with severe combined immunodeficiency disease, J Clin Invest, Vol.