Acute Promyelocytic Leukemia: Pathophysiology, Diagnosis and Clinical Management

Area of Science:

• Hematology
• Oncology
• Molecular Biology

Background:

• Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia.
• Characterized by the t(15;17) translocation and PML::RARA fusion gene.
• Early mortality is high due to coagulopathy.

Purpose of the Study:

• Summarize recent insights into APL pathophysiology.
• Review diagnostic approaches and management strategies.
• Provide evidence-based recommendations for clinical practice.

Main Methods:

• Comprehensive literature review.
• Focus on molecular mechanisms, coagulopathy, diagnosis, and therapy.
• Analysis of evidence-based recommendations.

Main Results:

• PML::RARA oncoprotein disrupts myeloid differentiation and apoptosis resistance.
• APL coagulopathy involves tissue factor, cancer procoagulant, cytokines, and hyperfibrinolysis.
• Diagnosis integrates morphology, immunophenotyping, coagulation, and molecular tests.

Conclusions:

• APL is highly curable with early recognition and targeted therapy.
• Prompt all-trans-retinoic acid (ATRA) and hemostatic support reduce early mortality.
• Minimal residual disease monitoring can further improve outcomes.