PDF(Pubmed)
Abstract：
Differentiation syndrome (DS) is a frequent and potentially life-threatening clinical syndrome first recognized with the advent of targeted therapeutics for acute promyelocytic leukemia (APL). DS was subsequently observed more broadly with targeted therapeutics for acute myeloid leukemia (AML). DS is typically characterized by fever, dyspnea, hypotension, weight gain, pleural or pericardial effusions, and acute renal failure. The incidence in patients with APL ranges from 2 to 37%, with the wide variation likely attributed to different diagnostic criteria, use of prophylactic treatment, and different treatment regimens. Treatment with corticosteroids +/- cytoreductive therapy should commence as soon as DS is suspected to reduce DS-related morbidity and mortality. The targeted anti-leukemic therapy should be discontinued in patients with severe DS. Here, we discuss the pathogenesis of DS, clinical presentations, diagnostic criteria, management strategies, and implementation of prospective tracking on clinical trials.
摘要：
分化综合征（DS）是一种常见且可能危及生命的临床综合征，最初是随着急性早幼粒细胞白血病（APL）的靶向治疗方法的出现而认识到的。随后用急性髓性白血病(AML)的靶向治疗剂更广泛地观察到DS。DS的典型特征是发烧，呼吸困难,低血压,体重增加,胸膜或心包积液，和急性肾衰竭.APL患者的发病率为2％至37％，与广泛的差异可能归因于不同的诊断标准，使用预防性治疗，和不同的治疗方案。一旦怀疑DS可降低DS相关的发病率和死亡率，就应开始使用皮质类固醇+/-细胞减灭剂治疗。严重DS患者应停止靶向抗白血病治疗。这里,我们讨论了DS的发病机制，临床表现,诊断标准,管理策略，并对临床试验实施前瞻性跟踪。
