Methods: Three patients with AT and poor antibody response to specific antigens presented for evaluation. One patient was a 17 year old male with active sarcoid skin disease, advanced neurological degeneration, chronic EBV and respiratory infections. The second patient was a 17 year old female with AT and scoliosis. The third patient was a 21 year old with AT and recurrent warts. All patients had poor response to protein and polysaccharide vaccines.
Results: The first patient had a pre-treatment IgG level of 293 mg/dl. He was started on IVIG 500 mg/kg/month. He received three doses of IVIG and transitioned to weekly subcutaneous gamma globulin therapy at a dose of 125 mg/kg/week, his one month post-treatment level was 633 mg/dl. The second patient had a pre-treatment IgG level of 118 mg/dl. She received five doses of IVIG at 500 mg/kg and transitioned to a weekly subcutaneous dose of 125 mg/kg/week with a one month post-treatment level of 906 mg/dl. Both patients tolerated the infusions well without pre-medication. The third patient had normal IgG of 1121 mg/dl, with poor response to vaccines. She was started on subcutaneous dose of 125 mg/kg/week. None of the patients had any skin changes or significant site reactions to SCIg.
Conclusions: Young adult patients with Ataxia Telangiectasia and poor vaccine response tolerated use of 20% subcutaneous gamma globulin.