All-trans retinoic acid syndrome

Last revised by Daniel J Bell on 27 Apr 2022

All-trans retinoic acid (ATRA) syndrome, more recently known as differentiation syndrome 8, is a condition that can occur with patients with acute promyelocytic leukemia who are on therapeutic all-trans-retinoic acid (ATRA).

All-trans retinoic acid (ATRA) is a normal constituent of plasma that is derived physiologically by intracellular oxidation of plasma retinol that has been absorbed from the intestines. ATRA is also used as an accepted therapy for acute promyelocytic leukemia. ATRA can differentiate acute promyelocytic leukemia blasts into mature granulocytes.

It is characterized by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary infiltrates noted on chest radiography.

Will depend on the system involved although many of the features are described in relation to the lungs.

Although the chest radiographic features are not specific, in the context of an appropriate clinical history, they may aid in early recognition of the ATRA syndrome. Recognized chest radiographic features that can be part of the ATRA syndrome include: 

In addition, chest radiography may show nodules and consolidation. 

CT features may not be specific and the clinical context is extremely useful in image interpretation. According to one study, the most consistent CT chest findings were small, irregular peripheral nodules in the lungs along with pleural effusions 5.

Early intervention with high dose corticosteroids is thought to abort progression. While most patients do well the occurrence of ATRA syndrome was associated with lower event-free survival.

It was first described in 1991 by Frankel et al. 1-3,7.

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Cases and figures

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