2003
DOI: 10.1080/08880010390243086
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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 includ… Show more

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Cited by 14 publications
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“…In DKC, circulating lymphocytes and fibroblasts exhibit an increased in vitro sensitivity to radiation and alkylating agents. 10 Therefore, this suggested that HSCT using myeloablative conditioning regimens in DKC patients would be associated with high regimen-related toxicity. 10 For this reason, RIC regimens are strongly recommended in HSCT for DKC patients.…”
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confidence: 99%
“…In DKC, circulating lymphocytes and fibroblasts exhibit an increased in vitro sensitivity to radiation and alkylating agents. 10 Therefore, this suggested that HSCT using myeloablative conditioning regimens in DKC patients would be associated with high regimen-related toxicity. 10 For this reason, RIC regimens are strongly recommended in HSCT for DKC patients.…”
mentioning
confidence: 99%
“…An increased in vitro sensitivity to radiation and alkylating agent in circulating lymphocytes and fibroblasts was demonstrated in a 7-year-old HHS boy. 8 This was correlated to telomere shortening. BMT using conventional conditioning regimes in DC patients have been associated with high mortality, particularly related to pulmonary and vascular complications.…”
mentioning
confidence: 99%
“…BMT using conventional conditioning regimes in DC patients have been associated with high mortality, particularly related to pulmonary and vascular complications. 8 This highlights the importance of making a correct diagnosis with the subsequent use of a RIC regimen.…”
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confidence: 99%
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