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Successful allogeneic stem cell transplant for leukocyte adhesion deficiency using an adjusted busulfan‐containing regimen
Author(s) -
Yoshimoto Akira,
Hashii Yoshiko,
Kashiwagi Hiroko,
Koizumi Makoto,
Tokimasa Sadao,
Fujisaki Hiroyuki,
Ohta Hideaki,
Hanai Naomi,
Ozono Keiichi,
Hara Junichi
Publication year - 2007
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2007.00723.x
Subject(s) - medicine , busulfan , leukocyte adhesion deficiency , regimen , transplantation , hematopoietic stem cell transplantation , conditioning regimen , stem cell , surgery , cd18 , integrin , receptor , biology , genetics
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.