论文部分内容阅读
慢性粒细胞性白血病(慢粒)是由于多能干细胞发生赘生性变而产生的一种单克隆骨髓增殖性疾病。本病大概于1845年由Graigie等首先发现。1960年Nowell等在费城首次于本病患者血细胞中发现1个异常的微小染色体,称为Ph′染色体。临床特点:慢粒患者通常有不适、发热、夜汗、消瘦、腹胀、巨脾、白细胞增多伴嗜酸、嗜碱粒细胞绝对数增多,白细胞碱性磷酸酶活力减低或缺如,出现Ph′染色体等综合表现,故一般较易确诊。在本病病程中,约30~40%患者出现骨髓纤维化。实验证明,成纤维细胞不是恶性(Ph′阳性)克隆演变而来的,骨纤的原因很可能是由于异常的造血细胞产生一种促纤维母细胞化因子所致。慢粒确诊后平均3年内将从相对良性的慢性期进入加速恶化期或原始细胞危象期。此时周围血和骨髓原始细胞数不断增加、进行性贫血、血小板减少,对治疗不显效,骨髓外可发生原粒细胞肿瘤。
Chronic myelogenous leukemia (CML) is a monoclonal myeloproliferative disorder that results from the neoplastic transformation of pluripotent stem cells. The disease was first discovered by Graigie et al in 1845. In 1960, Nowell et al. First found an abnormal microchromosome in the blood cells of patients with this disease in Philadelphia, called the Ph \'chromosome. Clinical features: CML patients usually have discomfort, fever, night sweat, weight loss, bloating, splenomegaly, leukocytosis with acidophilic, basophil absolute number increased, leucocyte alkaline phosphatase activity decreased or absent, there Ph \' Chromosomes and other comprehensive performance, it is generally easier to diagnose. In the course of the disease, about 30 to 40% of patients with myelofibrosis. Experiments have shown that fibroblasts do not evolve from malignant (Ph \'positive) clones that are most likely due to aberrant hematopoietic cells producing a fibroblast-like factor. Chronic granulomatosis after diagnosis within an average of three years from the relatively benign chronic phase into accelerated deterioration or primal cell crisis. At this point the number of peripheral blood and bone marrow primitive cells continue to increase, anemia, thrombocytopenia, the treatment is not effective, can occur in bone marrow neoplasm.