Rogers Jane E, Yang Daisy
UT MD Anderson Cancer Center, Houston, TX 77030, USA.
J Oncol Pharm Pract. 2012 Mar;18(1):109-14. doi: 10.1177/1078155211399163. Epub 2011 Mar 1.
To review the pathophysiology, risk factors, and management of differentiation syndrome (DS) associated with acute promyelocytic leukemia (APL).
A MEDLINE search was conducted (1977-November 2010) using the terms APL, DS, all-trans retinoic acid (ATRA), retinoic acid syndrome, and arsenic trioxide (ATO).
English articles identified from the MEDLINE search were evaluated.
With ATRA, ATO, and chemotherapy, a complete remission is achievable for most newly diagnosed APL patients. However, treatment with the differentiating agents, ATRA and ATO, can lead to the development of DS. Signs and symptoms of this syndrome include hyperleukocytosis and cardiorespiratory compromise. Severe complications can develop, if DS is not recognized early and treated promptly with corticosteroids. In addition, patients with a high white blood cell count at diagnosis may benefit from prophylactic steroids.
Early recognition and prompt initiation of corticosteroids are key factors in the management of DS. Healthcare professionals need to be familiar with this complication which can arise from differentiation agents.
回顾与急性早幼粒细胞白血病（APL）相关的分化综合征（DS）的病理生理学、危险因素及管理。
使用术语APL、DS、全反式维甲酸（ATRA）、维甲酸综合征及三氧化二砷（ATO）对MEDLINE进行检索（1977年至2010年11月）。
对从MEDLINE检索中识别出的英文文章进行评估。
使用ATRA、ATO及化疗，大多数新诊断的APL患者可实现完全缓解。然而，使用分化剂ATRA和ATO进行治疗可导致DS的发生。该综合征的体征和症状包括白细胞增多和心肺功能不全。如果DS未被早期识别并及时用皮质类固醇治疗，可能会出现严重并发症。此外，诊断时白细胞计数高的患者可能从预防性使用类固醇中获益。
早期识别并及时使用皮质类固醇是DS管理的关键因素。医疗保健专业人员需要熟悉这种可能由分化剂引起的并发症。
