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Acute promyelocytic leukaemia:a review
Simrit Parmar1, Martin S Tallman
1Division of Hematology-Oncology, Northwestern University Feinberg School of Medicine, Robert H Lurie Comprehensive Cancer Center, 676 North St. Clair, Suite 850, Chicago, IL 60611, USA.
Expert Opinion on Pharmacotherapy
|July 25, 2003
Summary
Acute promyelocytic leukemia (APL) is a curable leukemia. All-trans retinoic acid (ATRA) combined with chemotherapy offers high remission rates and survival, but carries risks like retinoic acid syndrome.
Area of Science:
- Hematology
- Oncology
- Molecular Biology
Background:
- Acute promyelocytic leukemia (APL) is defined by the PML-RAR-alpha fusion gene.
- APL presents with hemorrhagic diathesis and Auer rods in blasts.
- Poor prognostic factors include advanced age, high WBC, low platelets, and CD56 expression.
Purpose of the Study:
- To review the current understanding and treatment strategies for acute promyelocytic leukemia (APL).
- To highlight the impact of all-trans retinoic acid (ATRA) in APL treatment.
- To discuss challenges and future directions in APL management.
Main Methods:
- Review of literature on APL pathogenesis, clinical presentation, and treatment modalities.
- Analysis of the efficacy and toxicity of ATRA-based induction and maintenance therapies.
- Evaluation of treatment options for relapsed and refractory APL, including arsenic trioxide and stem cell transplantation.
Main Results:
- ATRA induces differentiation of leukemic blasts, leading to rapid resolution of coagulopathy and improved survival.
- Concurrent ATRA and chemotherapy achieve high complete remission rates.
- Retinoic acid syndrome (RAS) is a significant toxicity associated with ATRA treatment.
Conclusions:
- ATRA combined with chemotherapy is a highly effective induction regimen for APL.
- Maintenance therapy with ATRA is crucial after achieving complete remission.
- Arsenic trioxide is effective for relapsed APL, and stem cell transplantation may benefit select patients.