Dastugue N, Demur C, Pris F, Bugat R, Attal M, Bourrouillou G, Colombies P
Laboratoire Central d'Hématologie et de Génétique, C.H.U. Purpan-Toulouse, France.
Cancer Genet Cytogenet. 1988 Feb;30(2):253-9. doi: 10.1016/0165-4608(88)90192-6.
A patient developed a secondary blood disorder 7 years after radiotherapy for a gastric lymphoma. The initial myelodysplastic syndrome evolved to a myeloproliferative phase with transient polycythemia, progressive thrombocythemia, and hyperleukocytosis. Chromosome analysis performed in the terminal phase showed del(5)(q13q31),t(9;22)(q34;q11), and a complex rearrangement involving chromosomes #2 and #3. A correlation between chromosomal abnormalities and hematologic findings could be established. In this case, we have assumed that the Philadelphia translocation is a late event, due to prior mutagen exposure, and its association with a common secondary abnormality (5q-), followed by a progressively developing myeloproliferative phase. Furthermore, the association of Ph and 5q- in a single clone seems to indicate that the same stem cell is affected by these two abnormalities.
一名患者在接受胃淋巴瘤放疗7年后出现继发性血液系统疾病。最初的骨髓增生异常综合征演变为骨髓增殖期，伴有短暂性红细胞增多、进行性血小板增多和白细胞增多。终末期进行的染色体分析显示del(5)(q13q31)、t(9;22)(q34;q11)以及涉及2号和3号染色体的复杂重排。可以确定染色体异常与血液学检查结果之间存在相关性。在这种情况下，我们认为费城染色体易位是一个晚期事件，是由于先前接触诱变剂所致，它与常见的继发性异常(5q-)相关，随后是逐渐发展的骨髓增殖期。此外，单个克隆中Ph和5q-的关联似乎表明同一干细胞受到这两种异常的影响。
