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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 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 Citation Map 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. Related Collections Transplantation Brief Reports Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Brief report Progressive neurologic dysfunctions 20 years after allogeneic bone marrow transplantation for Chediak-Higashi syndrome Marc Tardieu, Catherine Lacroix, Bénédicte Neven, Pierre Bordigoni, Geneviève de Saint Basile, Stéphane Blanche, and Alain Fischer From the Service de Neurologie, Département de Pédiatrie et Laboratoire de Neuropathologie, Hôpital Bicêtre, Assistance Publique-Hôpitaux de Paris, Paris, France; Service de Pédiatrie, Centre Hospitalier Universitaire, Nancy, France; and Unité d'Immunohématologie Pédiatrique et Institut National de la Santé et de Recherche Médicale (INSERM) U429, Hôpital Necker, Assistance Publique-Hôpitaux de Paris, Paris, France. Three patients with Chediak-Higashi syndrome underwent allogeneic bone marrow transplantation between the ages of 2 years 9 months 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 3 patients developed a neurologic deficit combining difficulty walking, loss of balance, and tremor. Neurologic evaluation demonstrated cerebellar ataxia and signs of peripheral neuropathy. Moderate axon loss and rarefaction of large myelinated fibers were observed on semithin sections of peripheral nerve. Cerebellar atrophy was detected by cerebral magnetic resonance imaging in 2 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 neurologic 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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