毛细胞白血病是一种罕见的血液系统恶性肿瘤,其特征是全血细胞减少症和明显的感染易感性。这种疾病患者的治疗取得了巨大进展,导致了高反应率和生存率的提高。然而,复发和适当的再治疗方法是持续的研究领域。该疾病及其有效治疗与免疫抑制有关。因为更多的病人正在接受替代方案的治疗,结果的比较将需要对响应的定义达成普遍一致,复发,和确定微小残留病的方法。国际公认的发展,可重复标准对于评估和比较临床试验以提供最佳护理至关重要.尽管在管理这些患者方面取得了成功,强烈建议继续参与现有的一线临床试验,特别是在复发情况下.毛细胞白血病基金会召开了一次国际会议,以提供共同的定义和结构来指导当前的管理。有大量的机会继续研究这种疾病。除了优化预防和管理严重感染风险的重要性外,对微小残留病和复发时的治疗进行有组织的评估为临床研究提供了充足的机会。最后,在越来越多的目前可控制的慢性疾病的幸存者中,对生活质量的学术评估值得进一步研究.针对这种疾病的共识指南的发展为持续改善患者的预后提供了框架。
Hairy cell leukemia is an uncommon hematologic malignancy characterized by pancytopenia and marked susceptibility to infection. Tremendous progress in the management of patients with this disease has resulted in high response rates and improved survival, yet relapse and an appropriate approach to re-treatment present continuing areas for research. The disease and its effective treatment are associated with immunosuppression. Because more patients are being treated with alternative programs, comparison of results will require general agreement on definitions of response, relapse, and methods of determining minimal residual disease. The development of internationally accepted, reproducible criteria is of paramount importance in evaluating and comparing clinical trials to provide optimal care. Despite the success achieved in managing these patients, continued participation in available clinical trials in the first-line and particularly in the relapse setting is highly recommended. The Hairy Cell Leukemia Foundation convened an international conference to provide common definitions and structure to guide current management. There is substantial opportunity for continued research in this disease. In addition to the importance of optimizing the prevention and management of the serious risk of infection, organized evaluations of minimal residual disease and treatment at relapse offer ample opportunities for clinical research. Finally, a scholarly evaluation of quality of life in the increasing number of survivors of this now manageable chronic illness merits further study. The development of
consensus guidelines for this disease offers a framework for continued enhancement of the outcome for patients.