Tertian G, Misrahi M, Diallo D, Mielot F, Leonard C, Salmeron S, Tchernia G
Laboratoire d'Hematologie, Centre Hospitalier et Universitaire de Bicetre, Le Kremlin-Bicetre, France.
Nouv Rev Fr Hematol (1978). 1995;37(4):245-7.
In the present study, we report the case of a patient displaying an abnormal chromatin clumping (ACC) syndrome, a rare disease which shares features with both myeloproliferative and myelodysplastic disorders. Although various non specific cytogenetic abnormalities have been observed in ACC, the presence of a Ph1 chromosome has not been reported. In our patient, despite a lack of Ph1, PCR analysis of blood and bone marrow samples revealed a BCR-ABL rearrangement. These results indicate that at least some cases of ACC syndrome could represent a form of Ph1-negative chronic myeloid leukaemia.
在本研究中，我们报告了一例表现出异常染色质凝聚（ACC）综合征的患者，这是一种罕见疾病，兼具骨髓增殖性疾病和骨髓增生异常综合征的特征。尽管在ACC中已观察到各种非特异性细胞遗传学异常，但尚未有关于Ph1染色体存在的报道。在我们的患者中，尽管缺乏Ph1，但对血液和骨髓样本进行的PCR分析显示存在BCR-ABL重排。这些结果表明，至少部分ACC综合征病例可能代表一种Ph1阴性慢性髓性白血病。
