Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study.
Neurofibromatosis and childhood leukaemia/lymphoma: a population-based UKCCSG study.
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DOI：
10.1038/bjc.1994.431
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发表时间：
1994-11
影响因子：
8.8
通讯作者：
Fitchett, M
中科院分区：
文献类型：
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作者：
Stiller, C A;Chessells, J M;Fitchett, M
众所周知，患有1型神经纤维瘤病（NF-1）的儿童患白血病的风险增加。我们进行了第一个详细的基于人群的研究白血病和非霍奇金淋巴瘤（NHL）与NF-1，以估计风险和阐明这些条件之间的关系。在17年的研究期间，NF-1患者中有5例慢性粒单核细胞白血病（CMML）（相对风险221; 95% CI 71-514），12例急性淋巴细胞白血病（ALL）（相对风险5.4; 95% CI 2.8-9.4）和5例NHL（相对风险10.0; 95% CI 3.3-23.4）。可对7例患者进行骨髓细胞遗传学检查。发现的具体异常是CMML儿童的单体21和ALL儿童的7 p+，17 p-。17 q（包括NF 1基因）未报告异常。CMML主要发生在男孩中，他们也有NF-1家族史。ALL和NHL更常见于无家族史的儿童。
There is a well-known raised risk of leukaemia in children with neurofibromatosis type 1 (NF-1). We carried out the first detailed population-based study of leukaemia and non-Hodgkin lymphoma (NHL) associated with NF-1 in order to estimate the risk and elucidate the relationship between these conditions. Over the 17 year study period there were five cases of chronic myelomonocytic leukaemia (CMML) in patients with NF-1 (relative risk 221; 95% CI 71-514), 12 cases of acute lymphoblastic leukaemia (ALL) (relative risk 5.4; 95% CI 2.8-9.4) and five cases of NHL (relative risk 10.0; 95% CI 3.3-23.4). Marrow cytogenetics could be reviewed for seven patients. Specific abnormalities found were monosomy 21 in a child with CMML and 7p+, 17p- in a child with ALL. No abnormalities were reported of 17q, which includes the NF1 gene. CMML occurred predominantly in boys, who also had a family history of NF-1. ALL and NHL were more often found in children with no previous family history.