Sandlund J T, Krance R, Pui C H, Hancock M, Crist W M, Filatov L V, Raimondi S C
Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101, USA.
Med Pediatr Oncol. 1997 Jan;28(1):6-8. doi: 10.1002/(sici)1096-911x(199701)28:1<6::aid-mpo2>3.0.co;2-p.
Certain constitutional chromosomal abnormalities increase the risk of malignancy and/or decrease treatment tolerance. We identified two patients with the XYY syndrome among a total of 444 male children with acute lymphoblastic leukemia who had complete cytogenetics studies. In both cases, the leukemic cell karyotype suggested a constitutional XYY abnormality that was confirmed in studies of lymphocytes obtained during remission. The incidence rate in our series is higher than that of the XYY syndrome in the general population (0.0045 vs. 0.001), but not significantly so. This finding and a literature review failed to confirm an increased frequency of the XYY syndrome among children with acute lymphoblastic leukemia. Both of our patients remain in remission 24 and 28 months, respectively, postdiagnosis. Their tolerance of intensive treatment, including high-dose methotrexate, suggests that the untoward treatment toxicity seen in patients with chromosomal abnormalities such as trisomy 21 does not extend to the XYY syndrome.
某些先天性染色体异常会增加患恶性肿瘤的风险和/或降低治疗耐受性。在总共444名接受了完整细胞遗传学研究的急性淋巴细胞白血病男童中，我们发现了两名患有XYY综合征的患者。在这两个病例中，白血病细胞的核型提示存在先天性XYY异常，这在缓解期获取的淋巴细胞研究中得到了证实。我们系列研究中的发病率高于一般人群中XYY综合征的发病率（0.0045对0.001），但差异无统计学意义。这一发现以及文献综述均未能证实急性淋巴细胞白血病患儿中XYY综合征的发生率增加。我们的两名患者在确诊后分别已缓解24个月和28个月。他们对包括大剂量甲氨蝶呤在内的强化治疗的耐受性表明，在染色体异常（如21三体综合征）患者中所见的不良治疗毒性并不适用于XYY综合征。
