Yoshimoto Akira, Hashii Yoshiko, Kashiwagi Hiroko, Koizumi Makoto, Tokimasa Sadao, Fujisaki Hiroyuki, Ohta Hideaki, Hanai Naomi, Ozono Keiichi, Hara Junichi
Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan.
Pediatr Transplant. 2007 Jun;11(4):453-5. doi: 10.1111/j.1399-3046.2007.00723.x.
LAD is a rare and fatal congenital disorder in which there is a defect of the leukocyte adhesion molecule (CD18) on neutrophils. Severe LAD results in frequent and potentially fatal infections. Although allogeneic HSCT is the only curative treatment for severe LAD, strategies for HSCT remain to be established since a high engraftment failure rate and severe persistent infection are still seen. We report a four-month-old boy with LAD who was successfully treated with allogeneic HSCT from an HLA-complete matched sibling following a conditioning regimen of pharmacokinetically adjusted individual busulfan doses.
