What is APL differentiation syndrome and how is it treated?

Updated: Jul 02, 2019
  • Author: Sandy D Kotiah, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

APL differentiation syndrome (also known as retinoic acid syndrome [RAS]) can occur within the first 21d of treatment and is characterized by the following:

  • Fever
  • Hypotension
  • Weight gain
  • Respiratory distress
  • Serositis with pleural or pericardial effusions
  • Hypoxemia
  • Radiologic infiltrates
  • Acute kidney injury
  • Hepatic dysfunction
  • Hyperleukocytosis is common, but leukocyte counts may be normal

RAS should be treated with dexamethasone 10 mg IV every 12h for ≥ 3d. The benefit of prophylactic corticosteroids for prevention of RAS remains uncertain, but prophylactic corticosteroids can be considered in patients with WBC count > 5-10 x 109 /L at presentation or in those whose WBC counts increase after the start of ATRA. [1]


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