all-trans-retinoic acid (ATRA)-induced differentiation syndrome

Etiology

• occurs in 25% of patients with acute promyelocytic leukemia who receive all-trans=retinoic acid (ATRA) or arsenic trioxide

Pathology

• release of cytokines from differenting promyelocytes leading to capillary leak syndrome

Clinical manifestations

• symptom onset occures within the 1st week of therapy (47%) or within the 3rd week of therapy (25%)
• dyspnea
• peripheral edema
• weight gain
• fever
• hypotension
• acute kidney injury
• variable occurence of
  • pericardial effusion
  • pleural effusion
  • pulmonary infiltrates

Radiology

• chest X-ray

Differential diagnosis

• pneumonia

Management

• dexamethasone
• treatment for pneumonia may be prudent

More general terms

• syndrome