All-trans retinoic acid syndrome
All-trans retinoic acid (ATRA) syndrome (more recently known as differentiation syndrome (DS)8) is a condition that can occur with patients with acute promyelocytic leukaemia who are on therapeutic all-trans-retinoic acid (ATRA).
All-trans-retinoic acid (ATRA) is a normal constituent of plasma. It 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 leukaemia. ATRA can differentiate acute promyelocytic leukaemia blasts into mature granulocytes.
It is characterised 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. Recognised chest radiographic features that can be part of the ATRA syndrome include:
- increased cardiothoracic ratio
- increased vascular pedicle width
- ground-glass opacities
- peribronchial cufﬁng
- septal lines
- pleural effusion(s)
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 findings were small, irregular peripheral nodules in the lungs along with pleural effusions 5.
Therapy and prognosis
Early intervention with high dose corticosteroids is thought to abort progression. While most patients do well the occurrence of ATRA syndrome was associated a lower event-free survival.
History and etymology
It was first described in 1991 by Frankel et al. 1-3,7.
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