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大约在90％的慢性粒细胞白血病(慢粒)患者可检出Ph~1染色体,其余10％的病例历来称为Ph~1阴性的慢粒。后者具有下述临床特点,如病程短、急变早、白细胞和血小板计数较低和尿溶菌酶增高等,但它是否为一个独立的病理学类型却一直没有澄清。鉴于FAB协作组提出的骨髓增生异常综合征(MDS)诊断分型的建议,作者对1966至1983年间芝加哥大学和其它单位诊断的25例Ph~1阴性慢粒,从细胞形态学角度作了重新评价,其中女性13例,男性12例,中数年龄68岁。作者细致地复看了他们原来的血和骨髓涂片。结果发现7例(28％)并非慢粒,而是其它的骨髓增生性疾病或反应性状态,如骨髓硬化伴髓样化生、真性红细胞增多症、嗜酸细胞增多症和类白血病反应等;17例修正诊断为MDS,其中难治
Ph ~ 1 chromosomes are detectable in about 90% of patients with chronic myeloid leukemia (CML), and the remaining 10% of cases have traditionally been referred to as Ph-1-negative CML. The latter has the following clinical features, such as short course, rapid change, low white blood cell and platelet count and increased urine lysozyme, but whether it is an independent pathological type has not been clarified. In view of the suggestion for the diagnosis of myelodysplastic syndromes (MDS) proposed by the FAB collaboration group, the authors reviewed 25 Ph-1 negative CGVs diagnosed by the University of Chicago and other institutions between 1966 and 1983 and reviewed the morphological aspects of the cells Evaluation, including 13 females, 12 males, the median age of 68 years. The author carefully reviewed their original blood and bone marrow smear. The results showed that 7 cases (28%) were not CML, but other myeloproliferative diseases or reactivity status such as myeloid sclerosis with myeloid metaplasia, polycythemia vera, eosinophilia and leukemia; 17 cases were diagnosed as MDS, of which refractory