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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.
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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.
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