Kitabayashi A, Miura I, Ito T, Takahashi M, Chubachi A, Niitsu H, Mamiya S, Miura A B
Third Department of Internal Medicine, Akita University School of Medicine.
Rinsho Ketsueki. 1993 Jun;34(6):733-7.
[Case 1] A 44-year-old female was referred to our hospital because of leukocytosis. The WBC count was 26400/microliters and NAP score 21. As Ph1 chromosome was detected, she was diagnosed as CML and treated with busulfan. Because of the rapid decrease of WBC, we stopped busulfan. Progressive pancytopenia and an increase of myeloblasts and promyeloblasts in the bone marrow was observed. We started vincristine and prednisolone therapy. Ph1 chromosome was not detectable and southern blot analysis did not show rearranged bands of M-bcr three years after the last therapy. [Case 2] A 74-year-old female was referred to our hospital by reason of leukocytosis and thrombocytosis. The WBC count was 22,500/microliters, the platelet 907,000/microliters, NAP score 53, and Ph1 chromosome was found. The diagnosis of CML was made, and she was treated with busulfan. The WBC rapidly fell to 1,900/microliters, when chromosome analysis revealed the presence of Ph1 negative clones (4/20). She was admitted due to thrombocytopenia and leukocytosis with the additional chromosome change of i (17q). Her peripheral blood and bone marrow pictures were consistent with blast crisis, and she died of cardiac tamponade. These two cases show the heterogeneity of CML patients, and also suggest the possibility that keeping the WBC count low may lead to a decrease of Ph1 positive clones.
[病例 1] 一名 44 岁女性因白细胞增多症转诊至我院。白细胞计数为 26400/微升,中性粒细胞碱性磷酸酶(NAP)评分 21。由于检测到 Ph1 染色体,她被诊断为慢性粒细胞白血病(CML),并接受白消安治疗。由于白细胞迅速减少,我们停用了白消安。观察到进行性全血细胞减少以及骨髓中原始粒细胞和早幼粒细胞增多。我们开始了长春新碱和泼尼松龙治疗。在最后一次治疗三年后,未检测到 Ph1 染色体,Southern 印迹分析也未显示 M-bcr 的重排条带。[病例 2] 一名 74 岁女性因白细胞增多症和血小板增多症转诊至我院。白细胞计数为 22500/微升,血小板计数为 907000/微升,NAP 评分 53,且发现 Ph1 染色体。诊断为 CML,她接受了白消安治疗。白细胞迅速降至 1900/微升,此时染色体分析显示存在 Ph1 阴性克隆(4/20)。她因血小板减少症和白细胞增多症入院,伴有额外的 i(17q)染色体改变。她的外周血和骨髓图像与急变期一致,最终死于心脏压塞。这两个病例显示了 CML 患者的异质性,也提示了维持低白细胞计数可能导致 Ph1 阳性克隆减少的可能性。