Yamamoto Katsuya, Nagata Kaoru, Morita Yuriko, Inagaki Koichi, Hamaguchi Hiroyuki
Department of Hematology, Musashino Red Cross Hospital, 1-26-1 Kyonan-cho, Musashino, Tokyo 180-8610, Japan.
Leuk Res. 2007 May;31(5):713-8. doi: 10.1016/j.leukres.2006.05.023. Epub 2006 Sep 18.
We describe here the first case of acute lymphoblastic leukemia (ALL) with an isodicentric Philadelphia [idic(Ph)] chromosome. A 35-year-old man was diagnosed as ALL because of the infiltration of CD10(+)CD19(+)CD33(+)CD34(+) lymphoblasts in the bone marrow and the expression of p190-type BCR/ABL fusion transcript. Chromosome analysis showed 45,XY,der(7;12)(q10;q10),der(9)t(9;22)(q34;q11),idic der(22)t(9;22)(q34;q11). The idic(Ph) chromosome was spindle-shaped and supposed to be formed by two Ph chromosomes joined at their q terminals, whereas idic(Ph) chromosomes in chronic myelogenous leukemia (CML) have been shown to be fused at the satellite regions of p arms. The results indicate that the structure of idic(Ph) chromosomes appears to be different between ALL and CML. The patient did not respond to any chemotherapy and could not achieve remission. This chromosome aberration in ALL may suggest poor prognosis as observed in some cases of CML. Furthermore, considering other three reported cases, der(7;12)(q10;q10) may be one of the recurrent translocations in ALL.
我们在此描述首例伴有等臂双着丝粒费城染色体[idic(Ph)]的急性淋巴细胞白血病（ALL）。一名35岁男性因骨髓中CD10(+)CD19(+)CD33(+)CD34(+)淋巴母细胞浸润及p190型BCR/ABL融合转录本表达而被诊断为ALL。染色体分析显示为45,XY,der(7;12)(q10;q10),der(9)t(9;22)(q34;q11),idic der(22)t(9;22)(q34;q11)。该idic(Ph)染色体呈纺锤形，推测由两条在q末端相连的费城染色体形成，而慢性髓性白血病（CML）中的idic(Ph)染色体已被证明是在p臂的卫星区域融合。结果表明，ALL和CML中idic(Ph)染色体的结构似乎有所不同。该患者对任何化疗均无反应，无法实现缓解。ALL中的这种染色体畸变可能提示预后不良，正如在某些CML病例中所观察到的那样。此外，考虑到其他三例报告病例，der(7;12)(q10;q10)可能是ALL中反复出现的易位之一。
