Lauta Vito Michele
Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine and Clinical Oncology, University of Bari Medical School, Policlinico, Piazza Giulio Cesare 11, 70124 Bari, Italy.
Med Oncol. 2003;20(2):95-116. doi: 10.1385/MO:20:2:95.
Chronic myelogenous leukemia (CML) consists of a clonal malignancy that arises from a pluripotent hematopoietic stem call. In most cases, neoplastic cells are characterized by the formation of a shortened chromosome 22 called the Philadelphia chromosome. It results from a reciprocal translocation between long arms of chromosomes 9 and 22. A rearranged gene (bcr-abl) is the consequence of this translocation, and it may be considered as the first step toward leukemic transformation. Conventional chemotherapy of CML in the chronic phase is unable to suppress the Ph+ leukemic clone. The treatment with the IFNalpha may induce an overall cytogenetic response rate of 40-50% of patients. Autografting for patients with CML in chronic phase may induce a 53% overall cytogenetic response rate with a duration of disease-free time and survival from the autograft ranging, respectively, from 4 to 24 mo and from 8 to 40 mo.
慢性粒细胞白血病（CML）是一种起源于多能造血干细胞的克隆性恶性肿瘤。在大多数情况下，肿瘤细胞的特征是形成一条缩短的22号染色体，即费城染色体。它是由9号和22号染色体长臂之间的相互易位导致的。这种易位产生了一个重排基因（bcr-abl），它可被视为白血病转化的第一步。慢性期CML的传统化疗无法抑制Ph+白血病克隆。α干扰素治疗可使40%至50%的患者产生总体细胞遗传学反应率。慢性期CML患者进行自体造血干细胞移植可使总体细胞遗传学反应率达到53%，自体造血干细胞移植后的无病生存期和生存期分别为4至24个月和8至40个月。
