b细胞慢性淋巴细胞白血病的治疗:现状和未来展望。

E Montserrat, F Bosch, C Rozman
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摘要

在过去的二十年中,在b细胞慢性淋巴细胞白血病(CLL)的生物学、自然史和预后方面取得了重要进展。此外,由于生存预后因素的确定以及新药物和治疗策略的可用性,CLL患者的治疗可能性也发生了变化。病情稳定的早期临床阶段(Binet A, Rai 0)患者有长期生存的可能性,除非病情进展,否则不应进行治疗。相比之下,大多数具有不良预后特征的患者,如晚期临床阶段(Binet B, C;Rai III, IV),弥漫性骨髓浸润或血淋巴细胞水平迅速升高,中位生存概率< 5年,需要治疗。嘌呤类似物非常有效。其中,氟达拉滨已成为标准治疗失败患者的治疗选择。嘌呤类似物单独或与其他药物联合作为一线治疗的作用正在研究中。某些情况(如自身免疫性细胞减少症、脾功能亢进)需要特殊的治疗方法(如皮质类固醇、脾切除术)。祖造血细胞移植也越来越多地应用于预后不良的年轻患者,值得进一步研究。由于这些进展,症状缓解不再是CLL治疗的唯一可能目标;在不久的将来可能会获得持续的缓解甚至治愈。
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Treatment of B-cell chronic lymphocytic leukaemia: current status and future perspectives.

In the last two decades, important advances have been made in the biology, natural history, and prognosis of B-cell chronic lymphocytic leukaemia (CLL). In addition, treatment possibilities for patients with CLL have changed as a result of the identification of prognostic factors for survival and the availability of new drugs and treatment strategies. Patients in the early clinical stages (Binet A, Rai 0) with stable disease have a probability of long survival and should not be treated unless the disease progresses. In contrast, most patients with poor prognostic features, such as an advanced clinical stage (Binet B, C; Rai III, IV), diffuse bone-marrow infiltration or rapidly increasing blood lymphocyte levels, have a median survival probability of < 5 years and require therapy. Purine analogues are highly effective. Among these, fludarabine has become the treatment of choice for patients failing standard therapies. The role of purine analogues either alone or in combination with other drugs as front-line therapy is being investigated. Certain situations (e.g. autoimmune cytopenias, hypersplenism) require special treatment approaches (e.g. corticosteroids, splenectomy). Transplants of progenitor haematopoietic cells are also increasingly performed and deserve further investigation in younger patients with poor prognostic features. As a result of these advances, symptoms palliation is no longer the only possible goal in CLL therapy; sustained remissions and even cures are likely to be obtained in the near future.

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