Kohno T, Amenomori T, Atogami S, Sasagawa I, Nakamura H, Kuriyama K, Tomonaga M
Department of Haematology, Atomic Disease Institute, Nagasaki University School of Medicine, Japan.
Br J Haematol. 1996 May;93(2):389-91. doi: 10.1046/j.1365-2141.1996.4931034.x.
We describe a patient with Philadelphia chromosome (Ph1)-positive acute lymphoblastic leukaemia (ALL) who developed it 2.5 years after being diagnosed with myelodysplastic syndrome (MDS). The patient initially had refractory anaemia (RA), but progressed to refractory anaemia with excess blasts (RAEB) 2 years later, that terminated in ALL. An immunophenotypic analysis of the lymphoblasts revealed CD10 and CD19 positive cells. The karyotype was normal 46,XY in RA phase, 46,XY,20q-during the RAEB phase, and 46,XY,t(9;22)(q34;q11),20q-during the ALL phase. Furthermore, p190 BCR-ABL mRNA was detected in the ALL blasts. These findings indicate that this ALL arose from the MDS clone through multiple cytogenetic evolutions, the final event of which was the acquisition of p190 BCR-ABL type Ph1.
我们描述了一名患有费城染色体（Ph1）阳性急性淋巴细胞白血病（ALL）的患者，其在被诊断为骨髓增生异常综合征（MDS）2.5年后发展为此病。该患者最初患有难治性贫血（RA），但2年后进展为难治性贫血伴原始细胞增多（RAEB），最终发展为ALL。对原始淋巴细胞的免疫表型分析显示CD10和CD19阳性细胞。核型在RA期为正常的46,XY，在RAEB期为46,XY,20q-，在ALL期为46,XY,t(9;22)(q34;q11),20q-。此外，在ALL原始细胞中检测到p190 BCR-ABL mRNA。这些发现表明，这种ALL是通过多次细胞遗传学演变从MDS克隆发展而来的，其最终事件是获得p190 BCR-ABL型Ph1。
