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Internal Medicine

Differentiation syndrome

Introduction

Differentiation syndrome, previously retinoic acid syndrome (RAS) or ATRA syndrome, is a potentially life-threatening complication in patients with acute promyelocytic leukemia (aPML) and associated with all-trans retinoic acid (ATRA) ‘tretinoin’ and arsenic trioxide use.


Clinical features

Common symptoms:

  • Respiratory distress & fever (> 80% cases)
  • Pulmonary infiltrates & pulmonary oedema (> 50% cases)
  • Weight gain (50% cases)
WJR-6-583-g001
Correlation between white blood cell count and chest X-ray. Note that the worsening of chest X-ray is directly linked with the fall of white blood cell due to the massive differentiation during the onset of differentiation syndrome. | Institute of Radiology, Department of Clinical and Biological Sciences of University of Turin, AOU S. Luigi Gonzaga, Regione Gonzole 10, 10043 Orbassano, Torino, Italy. WBC: White blood cells

Complications

  • Pleural effusion → respiratory distress → hypoxia
  • Volume overload: Pericardial effusionhypotensionrenal failure

Management

Best approach for management of RAS is early recognition and prompt administration of steroids at the first sign of unexplained dyspnea, fever, weight gain or pulmonary infiltrates.

  • Corticosteroids (Dexamethasone) (10 mg IV BD for ≥ 3 days)
  • Cessation of ATRA (depending on severity of RAS)
  • Addition of another antineoplastic agent (cytarabine) to ATRA (in patients with high WBC count)
    • Major disadvantage: Bone marrow suppression

Once RAS is resolved, ATRA can be reintroduced.

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