Department of Hematology, Oslo University Hospital, Aker, Trondheimsveien 235, NO 0514 Oslo, Norway.
In Vivo. 2011 May-Jun;25(3):431-7.
From 2005 to 2010, eight families with clustering of Hodgkin's lymphoma and other lymphoproliferative disorders were found: Hodgkin's lymphoma 9 cases, chronic lymphocytic leukemia 8, non-Hodgkin's lymphoma 3, and multiple myeloma 1 case. Seven cases of Hodgkin's lymphoma, all males, were seen in pleiotropic pairs of affected family members from two successive generations; two patients were sisters. Five of the seven pairs showed sign of anticipation. The 7 males with Hodgkin's lymphoma were found in 5 patrilineal pairs and 2 matrilineal pairs; 6 parent-offspring pairs and 1 uncle-nephew pair. In contrast to the matrilineal pairs, all patrilineal pairs, apart from one family with an only child, had healthy older siblings in accordance with a birth-order effect. The association among Hodgkin's lymphoma, males, and other lymphoproliferative disorders undoubtedly reflects genotypic traits of the susceptibility. A non-Mendelian segregation is discussed comprising genomic parental imprinting and incomplete penetrance susceptibility in both familial and solitary cases.
从 2005 年至 2010 年,共发现了 8 个霍奇金淋巴瘤和其他淋巴增殖性疾病聚集的家族:霍奇金淋巴瘤 9 例,慢性淋巴细胞白血病 8 例,非霍奇金淋巴瘤 3 例,多发性骨髓瘤 1 例。在来自两代的多对受累家庭成员中观察到 7 例霍奇金淋巴瘤,均为男性;其中 2 例为姐妹。7 对中的 5 对显示出预期的迹象。7 例男性霍奇金淋巴瘤存在于 5 个父系对和 2 个母系对中;有 6 对是父母-子女对,1 对是叔侄对。与母系对不同,除了一个只有一个孩子的家庭外,所有的父系对都有健康的年长兄弟姐妹,这符合出生顺序效应。霍奇金淋巴瘤、男性和其他淋巴增殖性疾病之间的关联无疑反映了易感性的基因型特征。讨论了一种非孟德尔遗传,包括家族性和孤立性病例中的基因组亲本印迹和不完全外显率易感性。