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 discharge 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 rutenely 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.