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Unrelated bone marrow transplantation using a reduced intensity-conditioning regimen in leukocyte adhesion deficiency

Bone Marrow Transplantation volume 37, pages 807–808 (2006)Cite this article

The severe phenotype of leukocyte adhesion deficiency (LAD) is a congenital disorder of the leukocyte function that is usually fatal in the first decade of life. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for this disease. Hematopoietic stem cell transplantation using a reduced intensity-conditioning (RIC) regimen might be useful for LAD because the HSCT with RIC is presumed to be sufficient for the establishment of a donor-derived immune reconstitution with less regimen-related toxicity, and patients treated by this modality usually have comorbidity. We herein describe the first case to undergo an unrelated donor bone marrow transplantation with RIC in a patient demonstrating LAD.

Patients with leukocyte adhesion deficiency type I (LAD-I) tend to suffer recurrent and life-threatening bacterial infections because of a lack of the leukocyte adherence and migration caused by a mutation in the gene encoding the beta-2 integrin, cluster of differentiation (CD)18, which is located on the long arm of chromosome 21. Such a mutation results in the absence or a reduced membrane expression of leukocyte-associated integrins: lymphocyte function-associated antigen 1 (CD11a/CD18), Mac-1 (CD11b/CD18) and p150-95 (CD11c/CD18).1 In patients with a severe phenotype, leukocytes are expressed at <1% of the normal levels of CD18, and at <10% of the normal levels in the moderate phenotype.2 Patients with the severe form of LAD-I usually die of infection before 2 years of age, while the moderate phenotype is associated with a more prolonged survival, although the quality of life is poor and death eventually occurs in most cases.3

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Authors and Affiliations

  1. Section of Pediatrics, National Kyushu Cancer Center, Fukuoka, Japan

    D Takahashi, Y Nagatoshi, Y Saito, J Inagaki, J Nagayama & Y Shinkoda

  2. Department of Pediatrics, Tokuyama Central Hospital, Tokuyama, Japan

    K Fujita

  3. Institute for Clinical Research, National Kyushu Cancer Center, Fukuoka, Japan

    J Okamura

Authors
  1. D Takahashi
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  2. Y Nagatoshi
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  3. Y Saito
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  4. J Inagaki
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  5. J Nagayama
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  6. Y Shinkoda
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  7. K Fujita
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  8. J Okamura
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Correspondence to Y Nagatoshi.

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Takahashi, D., Nagatoshi, Y., Saito, Y. et al. Unrelated bone marrow transplantation using a reduced intensity-conditioning regimen in leukocyte adhesion deficiency. Bone Marrow Transplant 37, 807–808 (2006). https://doi.org/10.1038/sj.bmt.1705336

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