Montserrat E, Bosch F, Rozman C
Postgraduate School of Hematology, Farreras Valentí, Department of Medicine, University of Barcelona, Hospital Clinic, Spain.
Ann Oncol. 1997;8 Suppl 1:93-101.
B-cell chronic lymphocytic leukemia (CLL) is a highly common form of leukemia characterized by the accumulation of long-lived, functionally inactive, mature appearing neoplastic B lymphocytes. In addition, immune disturbances such as hypogammaglobulinemia and autoimmune phenomena (particularly, autoimmune hemolytic anemia) are frequently found in CLL patients [1-2]. The etiology of CLL is unknown. In contrast with other leukemias, there is no relationship between CLL and exposure to radiation or other cytotoxic agents. A genetic basis is highly likely since there are differences in the incidence of CLL in different countries (e.g., CLL accounts for 30%-40% of all the leukemias in Western countries as compared to 5%-10% in Asian countries) and the risk of contracting CLL is higher among persons with first-degree relatives with the disease [3]. Because the incidence of CLL increases with age and the longer life expectancy of the general population, the age of patients at diagnosis is increasing. The median age at diagnosis is now about 70 years, with only one-third of the patients being less than 60 years of age. In the majority of the series, males predominate over females in a proportion of 1.5/1. The prognosis of patients with CLL is variable. However, clinical stages and other prognostic factors allow the individual risk of each patient to be assessed very accurately, which is useful for making treatment decisions. In the past two decades, significant progress has been made in CLL [4-10]. This review summarizes recent advances in the biology, diagnosis, and therapy of CLL.
B细胞慢性淋巴细胞白血病(CLL)是一种非常常见的白血病形式,其特征是长寿、功能不活跃、外观成熟的肿瘤性B淋巴细胞积聚。此外,CLL患者经常出现免疫紊乱,如低丙种球蛋白血症和自身免疫现象(特别是自身免疫性溶血性贫血)[1-2]。CLL的病因尚不清楚。与其他白血病不同,CLL与辐射或其他细胞毒性药物的接触无关。由于不同国家CLL的发病率存在差异(例如,在西方国家,CLL占所有白血病的30%-40%,而在亚洲国家为5%-10%),且一级亲属患有该病的人患CLL的风险更高,因此很可能存在遗传基础[3]。由于CLL的发病率随年龄增长以及普通人群预期寿命的延长而增加,患者的诊断年龄也在上升。目前诊断时的中位年龄约为70岁,只有三分之一的患者年龄小于60岁。在大多数系列研究中,男性与女性的比例为1.5/1。CLL患者的预后各不相同。然而,临床分期和其他预后因素可以非常准确地评估每个患者的个体风险,这有助于做出治疗决策。在过去二十年中,CLL领域取得了重大进展[4-10]。本综述总结了CLL在生物学、诊断和治疗方面的最新进展。