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: 

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.

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.

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

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Article information

rID: 17323
Section: Syndromes
Tag: cases, cases
Synonyms or Alternate Spellings:
  • Differentiation syndrome
  • All trans retinoic acid (ATRA) syndrome
  • ATRA syndrome
  • All-trans-retinoic acid syndrome
  • All-trans-retinoic acid (ATRA) syndrome
  • Differentiation syndrome (DS)
  • Retinoic acid syndrome

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