Department of Internal Medicine III, Ulm University, Ulm 89081, Germany.
Hematol Oncol Clin North Am. 2013 Apr;27(2):289-301. doi: 10.1016/j.hoc.2013.01.008.
Treatment of chronic lymphocytic leukemia has greatly advanced in the past few years since introduction of the fludarabine/cyclosphosphamide/rituximab regimen as first-line therapy. Nevertheless, 17p deletion represents a challenge because conventional treatment does not provide satisfactory results. 17p deletion and TP53 mutation are the major factors accounting for rapid disease progression, poor response to therapy, early relapse, and short survival. Allogeneic stem cell transplantation harbors curative potential but also considerable morbidity and mortality. Novel agents acting independently of the p53 signaling pathway, with favorable side-effect profiles, are promising. This review summarizes up-to-date knowledge about 17p deletion and the spectrum of treatment options.
近年来,随着氟达拉滨/环磷酰胺/利妥昔单抗方案作为一线治疗药物的引入,慢性淋巴细胞白血病的治疗有了很大的进展。然而,17p 缺失仍然是一个挑战,因为常规治疗并不能提供满意的结果。17p 缺失和 TP53 突变是导致疾病快速进展、治疗反应不佳、早期复发和生存期短的主要因素。异基因造血干细胞移植具有治愈潜力,但也有相当大的发病率和死亡率。作用于 p53 信号通路以外的新型药物,具有良好的副作用谱,具有广阔的应用前景。本文综述了最新的关于 17p 缺失和治疗选择的知识。