Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient

Radhia M'kacher; Veronique Laithier; Alexandre Valent; Francois Delhommeau; Dominique Violot; Eric Deutsch; Julien Dossou; Nadine Béron-Gaillard; Theodore Girinsky; Jean Bourhis; Patrice Carde; Alain Bernheim; Claude Parmentier

doi:10.1080/08880010390243086

Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient

Radhia M'kacher, Veronique Laithier, Alexandre Valent, Francois Delhommeau, Dominique Violot, Eric Deutsch, Julien Dossou, Nadine Béron-Gaillard, Theodore Girinsky, Jean Bourhis, Patrice Carde, Alain Bernheim, Claude Parmentier

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Résumé

Hoyeraal-Hreidarsson syndrome (HHS) is a severe infantile variant of X-linked dyskeratosis congenita (DC). The authors report evidence of increased in vitro sensitivity to radiation and alkylating agent in circulating lymphocytes and fibroblasts obtained from a 7-year-old boy with HHS. A major telomere shortening was also found (3 kb) as compared to healthy donors (10 kb). The standard treatments, chemotherapy regimens, and radiation therapy were not possible. The data suggest that conditioning regimens including TBI should not be used when a bone marrow transplantation procedure is planned in these patients.

langue originale	Anglais
Pages (de - à)	651-656
Nombre de pages	6
journal	Pediatric Hematology and Oncology
Volume	20
Numéro de publication	8
Les DOIs	https://doi.org/10.1080/08880010390243086
état	Publié - 1 janv. 2003

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10.1080/08880010390243086

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M'kacher, R., Laithier, V., Valent, A., Delhommeau, F., Violot, D., Deutsch, E., Dossou, J., Béron-Gaillard, N., Girinsky, T., Bourhis, J., Carde, P., Bernheim, A., & Parmentier, C. (2003). Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient. Pediatric Hematology and Oncology, 20(8), 651-656. https://doi.org/10.1080/08880010390243086

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title = "Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient",

abstract = "Hoyeraal-Hreidarsson syndrome (HHS) is a severe infantile variant of X-linked dyskeratosis congenita (DC). The authors report evidence of increased in vitro sensitivity to radiation and alkylating agent in circulating lymphocytes and fibroblasts obtained from a 7-year-old boy with HHS. A major telomere shortening was also found (3 kb) as compared to healthy donors (10 kb). The standard treatments, chemotherapy regimens, and radiation therapy were not possible. The data suggest that conditioning regimens including TBI should not be used when a bone marrow transplantation procedure is planned in these patients.",

keywords = "Bone marrow transplantation, Hoyeraal-Hreidarsson syndrome, Radiation sensitivity, Telomere length",

author = "Radhia M'kacher and Veronique Laithier and Alexandre Valent and Francois Delhommeau and Dominique Violot and Eric Deutsch and Julien Dossou and Nadine B{\'e}ron-Gaillard and Theodore Girinsky and Jean Bourhis and Patrice Carde and Alain Bernheim and Claude Parmentier",

note = "Funding Information: Received 26 March 2003; accepted 8 July 2003. We are grateful to Tom J. Vulliamy (Imperial College School of Medicine, London) for the screening of DKC1 gene and for his suggestions. We thank Danielle Fenneux (Hopital Kremlin Bic{\^e}tre) for the telomere length investigation and Nicole Chavaudra and Bernard Clausse for their participation in this study. We also thank Ingrid Kuchenthal for preparing the manuscript. This work was supported by grants from the Gustave Roussy Institute (CRC 98-25 and CRC 2000-01) and Electricit{\'e} de France (EDF 98-04). Address correspondence to Dr. Radhia M{\textquoteright}kacher, Institut Gustave Roussy, 94 805 Villejuif, France. E-mail: [email protected]",

year = "2003",

month = jan,

day = "1",

doi = "10.1080/08880010390243086",

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M'kacher, R, Laithier, V, Valent, A, Delhommeau, F, Violot, D, Deutsch, E, Dossou, J, Béron-Gaillard, N, Girinsky, T, Bourhis, J, Carde, P, Bernheim, A & Parmentier, C 2003, 'Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient', Pediatric Hematology and Oncology, VOL. 20, Numéro 8, p. 651-656. https://doi.org/10.1080/08880010390243086

TY - JOUR

T1 - Sensitivity to radiation and alkylating agent of peripheral lymphocytes and fibroblasts in a Hoyeraal-Hreidarsson syndrome patient

AU - M'kacher, Radhia

AU - Laithier, Veronique

AU - Valent, Alexandre

AU - Delhommeau, Francois

AU - Violot, Dominique

AU - Deutsch, Eric

AU - Dossou, Julien

AU - Béron-Gaillard, Nadine

AU - Girinsky, Theodore

AU - Bourhis, Jean

AU - Carde, Patrice

AU - Bernheim, Alain

AU - Parmentier, Claude

N1 - Funding Information: Received 26 March 2003; accepted 8 July 2003. We are grateful to Tom J. Vulliamy (Imperial College School of Medicine, London) for the screening of DKC1 gene and for his suggestions. We thank Danielle Fenneux (Hopital Kremlin Bicêtre) for the telomere length investigation and Nicole Chavaudra and Bernard Clausse for their participation in this study. We also thank Ingrid Kuchenthal for preparing the manuscript. This work was supported by grants from the Gustave Roussy Institute (CRC 98-25 and CRC 2000-01) and Electricité de France (EDF 98-04). Address correspondence to Dr. Radhia M’kacher, Institut Gustave Roussy, 94 805 Villejuif, France. E-mail: [email protected]

PY - 2003/1/1

Y1 - 2003/1/1

N2 - Hoyeraal-Hreidarsson syndrome (HHS) is a severe infantile variant of X-linked dyskeratosis congenita (DC). The authors report evidence of increased in vitro sensitivity to radiation and alkylating agent in circulating lymphocytes and fibroblasts obtained from a 7-year-old boy with HHS. A major telomere shortening was also found (3 kb) as compared to healthy donors (10 kb). The standard treatments, chemotherapy regimens, and radiation therapy were not possible. The data suggest that conditioning regimens including TBI should not be used when a bone marrow transplantation procedure is planned in these patients.

AB - Hoyeraal-Hreidarsson syndrome (HHS) is a severe infantile variant of X-linked dyskeratosis congenita (DC). The authors report evidence of increased in vitro sensitivity to radiation and alkylating agent in circulating lymphocytes and fibroblasts obtained from a 7-year-old boy with HHS. A major telomere shortening was also found (3 kb) as compared to healthy donors (10 kb). The standard treatments, chemotherapy regimens, and radiation therapy were not possible. The data suggest that conditioning regimens including TBI should not be used when a bone marrow transplantation procedure is planned in these patients.

KW - Bone marrow transplantation

KW - Hoyeraal-Hreidarsson syndrome

KW - Radiation sensitivity

KW - Telomere length

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SN - 0888-0018

VL - 20

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EP - 656

JO - Pediatric Hematology and Oncology

JF - Pediatric Hematology and Oncology

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ER -