Hasle H, Olsen J H
Department of Paediatrics, Odense University Hospital, Denmark.
Br J Haematol. 1997 Apr;97(1):127-31. doi: 10.1046/j.1365-2141.1997.202664.x.
Familial myelodysplastic syndrome (MDS) has been claimed to account for as many as one third of children with MDS, especially among those showing monosomy 7. The present study is the first to provide population-based estimates of the risk of haematological and other malignancies in relatives of children with MDS. The study was extended to include children with acute myeloid leukaemia (AML) and chronic myeloid leukaemia (CML). The index group consisted of 46 children with MDS, 62 with AML, and eight with CML, which is thought to represent all myeloid leukaemias in Danish children, 1980-91. By linkage to the Central Population Register we identified parents (230), siblings (231), grandparents (151), aunts and uncles (132) and cousins (140). Information on the cancer incidence was obtained from the Danish Cancer Registry. 27 cancers were observed versus 26.7 expected (relative risk 1.0). Leukaemia in relatives was observed in only one family. None of 11 children with MDS and monosomy 7 had family members affected by leukaemia. We found no evidence of an increased overall risk of cancer in the relatives. The risk of familial MDS may be considerably lower than previously estimated.
据宣称，家族性骨髓增生异常综合征（MDS）在患有MDS的儿童中占比高达三分之一，尤其是在那些出现7号染色体单体的儿童中。本研究首次提供了基于人群的MDS患儿亲属发生血液系统及其他恶性肿瘤风险的评估。该研究范围扩大至包括急性髓系白血病（AML）和慢性髓系白血病（CML）患儿。索引组由46例MDS患儿、62例AML患儿和8例CML患儿组成，被认为代表了1980 - 1991年丹麦儿童中的所有髓系白血病病例。通过与中央人口登记处联动，我们确定了患儿的父母（230人）、兄弟姐妹（231人）、祖父母（151人）、叔伯姑姨（132人）和堂表亲（140人）。癌症发病率信息来自丹麦癌症登记处。观察到27例癌症病例，预期为26.7例（相对风险1.0）。仅在一个家庭中观察到亲属患白血病。11例患有MDS且有7号染色体单体的患儿中，没有家庭成员患白血病。我们没有发现亲属患癌总体风险增加的证据。家族性MDS的风险可能远低于先前的估计。
