All-trans retinoic acid syndrome
All-trans retinoic acid (ATRA) syndrome, more recently known as differentiation syndrome (DS) , 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.
Clinical presentation
It is characterized by fever, respiratory distress, pleural and pericardial effusion, weight gain, and pulmonary infiltrates noted on chest radiography.
Radiographic features
Will depend on the system involved although many of the features are described in relation to the lungs.
Plain radiograph
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:
- 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
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 .
Treatment 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 with lower event-free survival.
History and etymology
It was first described in 1991 by Frankel et al. .
See also
