Akashi K, Taniguchi S, Nagafuji K, Harada M, Shibuya T, Hayashi S, Gondo H, Niho Y
Harasanshin General Hospital, Fukuoka, Japan.
Leuk Res. 1993 Jul;17(7):549-55. doi: 10.1016/0145-2126(93)90083-w.
We report two cases of Philadelphia chromosome (Ph)-positive acute leukemia with definite myeloid markers. Ph was the sole chromosomal abnormality at presentation, and neither eosinophilia, basophilia, thrombocytosis nor hepatosplenomegaly was present. In both cases, Ph+ myeloblasts showed positive stain for myeloperoxidase and naphthol ASD chloroacetate esterase, which fulfilled the FAB criteria of acute myelogenous leukemia (AML). Ph+ myeloblasts co-expressed myeloid and B-lymphoid antigens (CD10, CD13, CD19 and CD33). In case 1, myeloblasts rearranged M-BCR, and the expression of M-BCR/ABL chimeric RNA was demonstrated by using the reverse transcription polymerase chain reaction (RT-PCR). They also clonally rearranged IGH. Ph clone disappeared on cytogenetic analysis in remission, and granulocytes in remission did not have rearranged M-BCR. In case 2, morphocytochemically distinct myeloid and lymphoid blast populations were seen. Myeloblasts and lymphoblasts were enriched > 96% as CD19-/CD33+ and CD19+/CD33- populations, respectively. Both of them possessed the identical rearrangement of IGH and M-BCR, indicating a common leukemic progenitor cell origin. Furthermore, m-BCR/ABL was detected in addition to M-BCR/ABL on RT-PCR. Accordingly, both cases were diagnosed as de novo Ph+ acute leukemia rather than as chronic myelogenous leukemia in blastic crisis. Their mixed B-lymphoid/myeloid characteristics strongly suggest that so-called 'Ph+ AML' is derived from Ph+ myeloid/B-lymphoid stem cells.
我们报告了2例具有明确髓系标志物的费城染色体（Ph）阳性急性白血病。Ph是初诊时唯一的染色体异常，且不存在嗜酸性粒细胞增多、嗜碱性粒细胞增多、血小板增多或肝脾肿大。在这2例病例中，Ph+原始粒细胞髓过氧化物酶和萘酚ASD氯乙酸酯酶染色呈阳性，符合急性髓系白血病（AML）的FAB标准。Ph+原始粒细胞共表达髓系和B淋巴细胞抗原（CD10、CD13、CD19和CD33）。在病例1中，原始粒细胞发生M-BCR重排，通过逆转录聚合酶链反应（RT-PCR）证实了M-BCR/ABL嵌合RNA的表达。它们还发生了IGH的克隆性重排。缓解期细胞遗传学分析显示Ph克隆消失，缓解期粒细胞未发生M-BCR重排。在病例2中，可见形态细胞化学上不同的髓系和淋巴系原始细胞群。原始粒细胞和原始淋巴细胞分别作为CD19-/CD33+和CD19+/CD33-细胞群富集>96%。它们都具有相同的IGH和M-BCR重排，表明有共同的白血病祖细胞起源。此外，RT-PCR检测除了M-BCR/ABL外还检测到了m-BCR/ABL。因此，这2例病例均被诊断为原发性Ph+急性白血病，而非慢性髓系白血病急变期。它们混合的B淋巴细胞/髓系特征强烈提示所谓的“Ph+ AML”源自Ph+髓系/B淋巴细胞干细胞。
