Bunin N, Nowell P C, Belasco J, Shah N, Willoughby M, Farber P A, Lange B
Division of Oncology, Children's Hospital of Philadelphia, PA 19104.
Cancer Genet Cytogenet. 1991 Jul 1;54(1):119-26. doi: 10.1016/0165-4608(91)90039-w.
Three children, two boys and one girl, with Down syndrome (DS) who presented with preleukemia and loss of all or part of chromosome 7 were studied. Initial presentation, with cytopenias and less than 25% blasts in the bone marrow, was between 13 and 30 months of age. Progression to acute nonlymphocytic leukemia occurred 1-8 months after initial presentation. The morphologic type was megakaryoblastic in two, and undifferentiated in one. Two children achieved remission with intensive therapy, and one continues in remission off therapy; the other child died in remission of accidental causes. The third child died of respiratory distress and leukemia after no intervention was chosen. These cases represent the first examples of chromosome 7 abnormalities associated with DS and leukemia, and suggest differences from the "monosomy 7" syndrome seen in children without DS.
对三名患有唐氏综合征(DS)且伴有白血病前期以及7号染色体全部或部分缺失的儿童(两名男孩和一名女孩)进行了研究。最初表现为血细胞减少且骨髓中原始细胞少于25%,发病年龄在13至30个月之间。初次发病后1至8个月进展为急性非淋巴细胞白血病。形态学类型两名患儿为巨核母细胞型,一名为未分化型。两名患儿通过强化治疗实现缓解,一名患儿在停止治疗后持续缓解;另一名患儿因意外原因在缓解期死亡。第三名患儿在未采取任何干预措施后死于呼吸窘迫和白血病。这些病例代表了与唐氏综合征和白血病相关的7号染色体异常的首个实例,并提示与非唐氏综合征患儿中所见的“单体7”综合征存在差异。