541 Intravenous Immunoglobulin in Leukocyte Adhesion Deficiency

BACKGROUND: Leukocyte adhesion deficiency (LAD) is a primary immunodeficiency disease (PID) caused by a defect in neutrophil adhesion, characterized by skin ulcers, poor wound healing and recurrent bacterial infection. Intravenous immunoglobulin (IVIG) is used to treat patients with PID, but in LAD is not rutinely used. Treatment consists in prompt antibiotic, G-CSF for chronic ulcers and the only definite therapy is bone marrow transplantation (BMT). We present the case of a child with LAD, who was treated with IVIG with a good response before BMT. METHODS: We present a case report of a 2 year-old male, second child of consanguineous parents (cousins 1st grade). His sister had omphalitis and umbilical abscess and died at 6 months with candidiasis and perianal infection. There were 6 episodes of infectious diseases from birth to 6 months: At 11 days of life presented with omphalitis. At 2 months, upper respiratory tract infection with poor response to antibiotics. At 4 months he presented with suppurative otitis media, and was transferred to our hospital with suspected immunodeficiency, with neutrophilia (up to 95900). He was treated with IV antibiotics, and after resolution with prophylactic antibiotics. At 6 months had gastroenteritis and 1 week later septic shock. Treatment with intravenous immunoglobulins (IVIG) was started. RESULTS: After IVIG was initiated there were only 6 episodes of infectious diseases from 6 months to 2 years, including in the cord blood stem cell transplantation (CBSCT) period: at 9 months, gastroenteritis; at 15 months balanosposthitis (ecthyma gangrenosum), at 17 months had cellulitis in the hand and buttocks and oral candidiasis. CBSCT was performed on February 2011, at 1 year 11 months, but didn't engraft. He was discharged with prophylactic antibiotics and cyclosporine. At 2 years he had catheter associated sepsis. Currently the patient is receiving monthly IVIG, fluconazol, TMP SMX, Acyclovir and in protocol for BMT and has remained stable. CONCLUSIONS: IVIG is not routinely used in LAD. In our case, monthly IVIG resulted in improvement with less infectious episodes. We suggest the use of IVIG as an adjuvant tool for the treatment of patients with LAD before BMT.