[Acute promyelocytic leukaemia]

Area of Science:

• Hematology
• Oncology
• Molecular Biology

Context:

• Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML).
• APL presents unique biological, clinical, and therapeutic challenges.
• Understanding APL's specific characteristics is vital for effective management.

Purpose:

• To describe the development, pathophysiology, diagnosis, and treatment of APL.
• To highlight the critical role of early intervention in APL management.
• To review current therapeutic strategies and their outcomes.

Summary:

• APL is caused by the t(15;17) translocation, producing the PML-RARA oncoprotein.
• Diagnosis relies on clinical suspicion confirmed by detecting the translocation.
• Severe coagulopathy and bleeding, particularly intracranial hemorrhage, are common at diagnosis.
• Early treatment with all-trans retinoic acid (ATRA) is essential to reduce early mortality.
• ATRA combined with chemotherapy and arsenic offers high long-term survival rates (>90%).
• Effective treatments exist for elderly patients with comorbidities.

Impact:

• Rapid initiation of ATRA therapy significantly reduces life-threatening hemorrhages and early mortality in APL.
• Timely and appropriate treatment leads to a very good prognosis and high rates of disease-free long-term survival.
• This approach ensures better outcomes for APL patients, including vulnerable elderly populations.