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Related Experiment Videos

[Retinoid receptors and hematologic diseases]

L Degos1

  • 1Service des maladies du sang, hôpital Saint-Louis, Paris.

La Revue Du Praticien
|May 15, 1994
PubMed
Summary

All trans retinoic acid induces remission in acute promyelocytic leukemia (APL) by promoting malignant cell differentiation. Combining this with chemotherapy improves long-term survival, despite relapses due to retinoic acid metabolism.

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Early death in acute promyelocytic leukemia (APL) in French centers: a multicenter study in 399 patients.

Leukemia·2014
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Revue d'epidemiologie et de sante publique·2008
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Current topics in microbiology and immunology·2007
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Late first relapses in APL treated with all-trans-retinoic acid- and anthracycline- based chemotherapy: the European APL group experience (APL 91 and APL 93 trials).

Leukemia·2006
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Extramedullary relapse in acute promyelocytic leukemia treated with all-trans retinoic acid and chemotherapy.

Leukemia·2005

Area of Science:

  • Hematology
  • Oncology
  • Molecular Biology

Context:

  • Acute promyelocytic leukemia (APL) is a rare subtype of myeloid leukemia.
  • APL is characterized by a specific t(15;17) chromosomal translocation.
  • Patients often present with a severe bleeding disorder.

Purpose:

  • To investigate the efficacy of all-trans retinoic acid (ATRA) in treating APL.
  • To understand the mechanism of ATRA-induced differentiation in APL cells.
  • To evaluate the role of ATRA in combination with chemotherapy for APL consolidation.

Summary:

  • All-trans retinoic acid (ATRA) effectively induces terminal differentiation of malignant promyelocytes in APL, leading to complete remission and resolution of bleeding diathesis.
  • Relapses occur due to accelerated ATRA catabolism, necessitating consolidation therapy.
  • The t(15;17) translocation creates a fusion gene (PML-RARα) that inhibits granulocyte maturation; high-dose ATRA appears to overcome this block, though the exact mechanism remains unclear.

Impact:

  • ATRA monotherapy achieves rapid remission in APL without aplastic anemia.
  • Combination therapy with ATRA and chemotherapy significantly enhances long-term survival rates for APL patients.
  • Understanding the molecular basis of ATRA's action in APL provides insights into targeted cancer therapy.

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