ACUTE MYELOID LEUKEMIA
CAT.# FP-005: PML/RARA GENE FUSION PROBE
IVD/RUO
BACKGROUND
Acute promyelocytic leukemia (APL) is a specific subtype of acute myeloid leukemia. In cytogenetics and molecular biology, APL has a characteristic t(15;17)(q22;21) translocation, forming a PML-RARA fusion gene. A large number of data indicate that patients carrying the PML-RARA fusion gene are predictive of sensitivity to ATRA therapy and good clinical efficacy.
PROBE DESCRIPTION
The two probes bind to the target detection site by in situ hybridization using an orange-red fluorescein-labeled PML probe and a green fluorescein-labeled RARA probe. Under normal conditions (the PML/RARA gene is not fused), it shows two orange-red signals and two green signals under a fluorescence microscope. When a fusion gene is present, the green and orange-red signals form a yellow fusion signal due to recombination.
CLINICAL SIGNIFICANCE
PML/RARA gene fusion is a hallmark of acute promyelocytic leukemia (APL). PML/RARA protein fusion inhibits the differentiation and maturation of promyelocytic cells by dominant negative inhibition, thereby blocking cell differentiation leading to sustained proliferation. All-trans retinoic acid (ATRA) and arsenic trioxide can target the degradation of PML/RARA fusion protein, restore the function of wild-type PML and RARA genes, relieve their inhibition of gene transcription, induce cell differentiation and apoptosis, and effectively treat APL. The combination of ATRA and chemotherapy can achieve a complete response rate of 90% to 95% of APL, and can achieve long-term survival of more than 70% of patients.
References
- Abe S, et al. (2008) Cancer Genet and Cytogenet 184: 44-7.
- Sanz MA, et al. (2009) Blood 113: 1875-91
