Klein U E, Schwarze E W, Schwalbe P, Tolksdorf M E
Dtsch Med Wochenschr. 1976 Jun 11;101(24):915-20. doi: 10.1055/s-0028-1104187.
Observations in six adult patients with leukaemic differential white counts, predominantly mature-celled, and with hepatosplenomegaly show that the mature-celled but fulminant (para-)neutrophil leukaemia must be differentiated from Ph1-positive chronic myeloid leukaemia. This (para-)neutrophil leukaemia is probably identical with the previously described atypical chronic myelosis of the adult, chronic myeloid leukaemia of childhood and the Pelger-like chronic myeloid leukaemia. Cardinal signs are a mature-celled differential count, short life expectancy (1 year), initial platelet deficiency, increased activity of granulocyte alkaline phosphatase, absence of Ph1-chromosome, and poor therapeutic response to busulfan. This curious and yet apparently not uncommon disease has been observed in the adult age group predominantly in men. The frequently high HbF level observed in juvenile chronic myeloid leukaemia could not be demonstrated in adults. Some of these neutrophil leukaemias are characterized by medullary fibrosis and terminal increase of immature blast cells (blast crises?) of which the diagnostic reliability is still disputed.
对6例患有白血病性白细胞分类计数、主要为成熟细胞且伴有肝脾肿大的成年患者的观察表明，成熟细胞型但暴发性的（类）中性粒细胞白血病必须与Ph1阳性慢性粒细胞白血病相鉴别。这种（类）中性粒细胞白血病可能与先前描述的成人非典型慢性粒细胞增多症、儿童慢性粒细胞白血病以及Pelger样慢性粒细胞白血病相同。主要体征为成熟细胞分类计数、预期寿命短（1年）、初始血小板缺乏、粒细胞碱性磷酸酶活性增加、无Ph1染色体以及对白消安治疗反应不佳。这种奇特但显然并不罕见的疾病主要在成年男性中观察到。在青少年慢性粒细胞白血病中常见的高HbF水平在成人中未得到证实。其中一些中性粒细胞白血病的特征是骨髓纤维化和末期未成熟母细胞增加（母细胞危象？），其诊断可靠性仍存在争议。
