Werner-Favre C, Wyss M, Cabrol C, Félix F, Guenin R, Laufer D, Engel E
Am J Med Genet. 1984 Jun;18(2):215-21. doi: 10.1002/ajmg.1320180205.
Bloom syndrome (BS) was diagnosed in a 7-year-old boy during hospitalization for acute lymphoblastic leukemia (ALL). The patient had most of the signs of BS along with some atypical manifestations: absence of telangiectases, obesity, and moderate mental retardation. Results of the cytogenetic studies were fully consistent with the diagnosis of BS: the occurrence of quadriradial figures and a very high incidence of sister-chromatid exchanges (SCE). This child's ALL was of non-B, non-T type with the presence, at the time of diagnosis, of a marrow clone including two markers. A Yq - chromosome was detected in about 10% of PHA-stimulated lymphocytes but neither in bone marrow cells nor in skin fibroblasts. This case is the fifth instance of ALL out of 104 registered cases of BS.
一名7岁男孩在因急性淋巴细胞白血病(ALL)住院期间被诊断出患有布卢姆综合征(BS)。该患者具有BS的大多数体征以及一些非典型表现:无毛细血管扩张、肥胖和中度智力发育迟缓。细胞遗传学研究结果与BS的诊断完全一致:出现四射体图形以及姐妹染色单体交换(SCE)的发生率非常高。这个孩子的ALL是非B、非T型,诊断时存在一个包含两个标志物的骨髓克隆。在约10%的PHA刺激淋巴细胞中检测到Yq - 染色体,但在骨髓细胞和皮肤成纤维细胞中均未检测到。该病例是104例已登记的BS病例中的第5例ALL病例。