741
Case Series of Tolerability of SCIg in Young Adults with Ataxia Telangiectasia
Monday, March 7, 2016
South Exhibit Hall H (Convention Center)
Vivian P. Hernandez-Trujillo, MD FAAAAI, William R. Blouin, MSN ARNP CPNP, Jose G. Calderon, MD
Rationale: Some patients with Ataxia Telangiectasia (AT) require gamma globulin therapy for hypogammaglobulinemia or poor specific antibody production.  Little information regarding tolerability of SCIg in AT patients, even with skin disease, is available.

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.