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Blood, 1 July 2005, Vol. 106, No. 1, pp. 40-42. Prepublished online as a Blood First Edition Paper on March 24, 2005; DOI 10.1182/blood-2005-01-0319. This Article Full Text (PDF) All Versions of this Article: 2005-01-0319v1 106/1/40    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Tardieu, M. Articles by Fischer, A. Search for Related Content PubMed PubMed Citation Articles by Tardieu, M. Articles by Fischer, A. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted January 25, 2005 Accepted March 2, 2005 Progressive neurological dysfunctions twenty years after allogeneic bone-marrow transplantation for Chediak-Higashi syndrome Marc Tardieu*, Catherine Lacroix, Benedicte Neven, Pierre Bordigoni, Genevieve de Saint Basile, Stephane Blanche, and Alain Fischer Service de Neurologie, Departement de Pediatrie et Laboratoire de Neuropathologie, Hopital Bicetre, Assistance Publique-Hopitaux de Paris, Le Kremlin Bicere, France Unite d'Immunohematologie Pediatrique, Paris, France Service de Pediatrie, Centre Hospitalier Universitaire, Nancy, France INSERM U429, Hopital Necker, Assistance Publique-Hopitaux de Paris, Paris, France Unite d'Immunohematologie Pediatrique, Paris, France; INSERM U429, Hopital Necker, Assistance Publique-Hopitaux de Paris, Paris, France * Corresponding author; email: marc.tardieu{at}bct.ap-hop-paris.fr. Three patients with Chediak-Higashi syndrome underwent allogeneic bone marrow transplantation between the ages of 2 9/12 and 7 years. The outcome was uneventful, with sustained mixed chimerism. No subsequent recurrent infections or hemophagocytic syndrome were observed. At the age of 22 to 24 years, these three patients developed a neurological deficit combining difficulty walking, loss of balance and tremor. Neurological evaluation demonstrated cerebellar ataxia and signs of peripheral neuropathy. Moderate axon loss and rarefaction of large myelinated fibers were observed on semi-thin sections of peripheral nerve. Cerebellar atrophy was detected by cerebral magnetic resonance imaging in two patients. We also reviewed the very long-term outcome of the other 11 patients with Chediak-Higashi syndrome who had received bone-marrow transplants at our center since 1981. All displayed neurological deficits or low cognitive abilities. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: W. Fiskus, M. Pranpat, M. Balasis, P. Bali, V. Estrella, S. Kumaraswamy, R. Rao, K. Rocha, B. Herger, F. Lee, et al. Cotreatment with Vorinostat (Suberoylanilide Hydroxamic Acid) Enhances Activity of Dasatinib (BMS-354825) against Imatinib Mesylate-Sensitive or Imatinib Mesylate-Resistant Chronic Myelogenous Leukemia Cells. Clin. Cancer Res., October 1, 2006; 12(19): 5869 - 5878. [Abstract] [Full Text] [PDF] T. R. Mahoney, Q. Liu, T. Itoh, S. Luo, G. Hadwiger, R. Vincent, Z.-W. Wang, M. Fukuda, and M. L. Nonet Regulation of Synaptic Transmission by RAB-3 and RAB-27 in Caenorhabditis elegans Mol. Biol. Cell, June 1, 2006; 17(6): 2617 - 2625. [Abstract] [Full Text] [PDF] J. Baselga Targeting Tyrosine Kinases in Cancer: The Second Wave. Science, May 26, 2006; 312(5777): 1175 - 1178. [Abstract] [Full Text] [PDF]

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