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Retinoic acid syndrome.

Debapriya Datta1, Daniel A Gerardi

  • 1Dept of Pulmonary and Critical Care Medicine, St Francis Hospital and Medical Center, 114, Woodland Street, Hartford, CT 06105, USA.

Connecticut Medicine
|November 19, 2003
PubMed
Summary

All-transretinoic acid (ATRA) treats acute promyelocytic leukemia (APL) but can cause Retinoic Acid Syndrome (RAS). Prompt recognition and treatment of RAS are crucial for patient outcomes.

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Area of Science:

  • Hematology
  • Oncology
  • Pharmacology

Background:

  • All-transretinoic acid (ATRA) is a Vitamin A derivative used to treat acute promyelocytic leukemia (APL).
  • Retinoic Acid Syndrome (RAS) is a known complication during ATRA treatment for APL.
  • RAS presents as a constellation of clinical signs and symptoms.

Observation:

  • Diagnosis of RAS in APL patients treated with ATRA requires at least three features: fever, dyspnea, weight gain, hypotension, renal failure, pulmonary infiltrates, pleural effusion, or pericardial effusion.
  • Pulmonary manifestations, particularly pulmonary edema, are the most frequent clinical presentation of RAS.
  • The incidence of RAS ranges from 6% to 27% among APL patients treated with ATRA.

Findings:

  • The exact pathogenesis of Retinoic Acid Syndrome remains incompletely understood.
  • RAS can be a life-threatening condition if not identified and managed promptly.
  • This report details a case of RAS in a young male patient with APL undergoing ATRA therapy.

Implications:

  • Early identification and management of RAS are critical for improving patient survival in APL.
  • Understanding RAS pathogenesis may lead to improved treatment strategies and preventative measures.
  • This case highlights the importance of vigilance for RAS in patients receiving ATRA for APL.

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