文章：

急性白血病中的分化综合征：从急性早幼粒细胞白血病到其他类型

Differentiation Syndrome in Acute Leukemia: APL and Beyond

原文发布日期：28 September 2023

DOI: 10.3390/cancers15194767

类型: Article

开放获取: 是

英文摘要：

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.

摘要翻译： 

分化综合征是一种常见且可能危及生命的临床综合征，最初在急性早幼粒细胞白血病靶向治疗问世时被发现。随后在急性髓系白血病靶向治疗中更广泛地观察到该综合征。其典型临床特征包括发热、呼吸困难、低血压、体重增加、胸腔或心包积液以及急性肾功能衰竭。APL患者中分化综合征的发生率为2%至37%，这种显著差异可能源于不同的诊断标准、预防性治疗措施及不同治疗方案的应用。一旦怀疑分化综合征，应立即开始皮质类固醇联合或不联合细胞减灭治疗，以降低相关发病率和死亡率。对于重症分化综合征患者，应暂停靶向抗白血病治疗。本文系统探讨了分化综合征的发病机制、临床表现、诊断标准、治疗策略及临床试验中前瞻性追踪的实施。

原文链接：

Differentiation Syndrome in Acute Leukemia: APL and Beyond