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Acute Promyelocytic Leukemia (APL)
Acute Promyelocytic Leukemia (APL)
Acute promyelocytic leukemia is a relatively rare and comprises about 7% to 8% of adult AML cases. Acute promyelocytic leukemia is usually seen in middle-aged people with a median age of 47 years. Acute promyelocytic leukemia occurs very rarely before the age of 20. [3].
(90% to 95%) of the cases, APL results from a t (15;17) (q22;q21) translocation resulting in the head to tail fusion of the promyelocytic leukemia (PML) gene, to RAR-alpha to generate two fusion genes, PML-RARalpha and a reciprocal RAR-alpha-PML (80%) that encode a protein, which functions as an aberrant retinoid receptor [3].
(90% to 95%) of the cases, APL results from a t (15;17) (q22;q21) translocation resulting in the head to tail fusion of the promyelocytic leukemia (PML) gene, to RAR-alpha to generate two fusion genes, PML-RARalpha and a reciprocal RAR-alpha-PML (80%) that encode a protein, which functions as an aberrant retinoid receptor [3].
Responsive to retinoid treatment
The PML/RARa protein heterodimerizes with the retinoid X receptor (RXR), the resulting PML/RARa-RXR complex binds to retinoic acid-responsive elements in target genes, resulting in cessation of myeloid differentiation at the promyelocytic stage. The excessive promyelocytes [3].
Acute promyelocytic leukemia is a medical emergency with a very high pre-treatment mortality. All-Trans Retinoic Acid (ATRA) is the mainstay in the treatment of acute promyelocytic leukemia and used in all modern regimens. [3]
ATO (arsenic trioxide) also induces differentiation of the malignant myeloid clone by dissociating the PML/RAR-alpha-RXR complex from the target genes and found to have a synergistic action with ATRA.[3].
==Gene Overview==
==Gene Overview==