Department of Leukemia, University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 428, Houston, TX 77030, USA.
Curr Hematol Malig Rep. 2011 Mar;6(1):36-46. doi: 10.1007/s11899-010-0069-3.
Chronic lymphocytic leukemia (CLL) is a lymphoproliferative disease with significant variation in disease progression, response to therapy, and survival outcome. Deletions of 17p or mutations of TP53 have been identified as one of the poorest prognostic factors, being predictive of short time for disease progression, lack of response to therapy, short response duration, and short overall survival. The treatment of patients with CLL has improved significantly with the development of chemoimmunotherapy, but this benefit was not pronounced in patients with 17p deletion. We compare various treatment strategies used in these patients, including FCR-like chemoimmunotherapy, alemtuzumab, other antibody combinations, or novel targeted therapies with promising results. Allogeneic stem cell transplantation offers the possibility for long-term disease control in these patients and should be considered early in younger, transplant-eligible patients. The current state of therapy is far from optimal and resources should be applied to studying therapeutic options for patients who have CLL with loss of p53 function.
慢性淋巴细胞白血病(CLL)是一种淋巴增殖性疾病,其疾病进展、对治疗的反应和生存结局存在显著差异。17p 缺失或 TP53 突变已被确定为预后最差的因素之一,其预示着疾病进展时间短、对治疗无反应、反应持续时间短和总生存期短。随着化疗免疫疗法的发展,CLL 患者的治疗有了显著改善,但这一益处并未在 17p 缺失的患者中明显体现。我们比较了这些患者使用的各种治疗策略,包括 FCR 样化疗免疫疗法、阿仑单抗、其他抗体组合或具有前景的新型靶向治疗。异基因造血干细胞移植为这些患者提供了长期疾病控制的可能性,在年轻、适合移植的患者中应尽早考虑。目前的治疗状况远非最佳,应将资源用于研究对丧失 p53 功能的 CLL 患者的治疗选择。