What is the translocation in acute promyelocytic leukemia?
What is the translocation in acute promyelocytic leukemia?
Abstract. Acute promyelocytic leukemia is characterized by a specific t(15;17) chromosomal translocation and a particular sensitivity to retinoic acid. The translocation fuses the PML gene to the retinoic acid receptor alpha (RAR alpha) gene resulting in the production of a PML-RAR alpha fusion protein.
What chromosome translocation is most commonly associated with APML?
Pathogenesis. Acute promyelocytic leukemia is characterized by a chromosomal translocation involving the retinoic acid receptor-alpha gene on chromosome 17 (RARA).
Is APML cancer curable?
Because of advances in diagnosis and treatment of this disease, APL is now considered the most curable form of adult leukemia. Cure rates of 90 percent have been reported from centers specializing in APL treatment.
What is PML RARA translocation?
Promyelocytic leukemia/retinoic acid receptor alpha or PML-RARA refers to an abnormal fusion gene sequence. It is a specific rearrangement of genetic material from two separate chromosomes (chromosomal translocation) and is associated with a specific type of leukemia.
How long can you live with APL leukemia?
APL is now considered a highly curable disease, with 2-year event-free survival rates of 75–84%. Early mortality is common in APL and is frequently related to hemorrhagic complications. Prior to ATRA therapy, early death (ED) related to hemorrhage occurred in up to 26% of cases.
How does ATRA work in APL?
All-Trans Retinoic Acid (ATRA) This drug, a vitamin A derivative, has become a standard component of induction therapy for APL. ATRA targets and eliminates the PML/RARα abnormality. This treatment causes a marked decrease in the concentration of leukemic blast cells in the marrow, and a remission frequently follows.
Is APML genetic?
Acute promyelocytic leukemia is not inherited but arises from a translocation in the body’s cells that occurs after conception.
Which type of leukemia is most curable?
While it is similar in many ways to the other subtypes, APL is distinctive and has a specific treatment regime. Treatment outcomes for APL are very good, and it is considered the most curable type of leukemia, with cure rates as high as 90%.
What triggers APL?
Causes. APL is caused by the uncontrolled proliferation of promyelocytes, a type of immature cell from the myeloid lineage of blood cells. The hallmark of APL is genetic alterations involving the retinoic acid receptor alpha (RARA) gene.
Can APML come back?
Acute promyelocytic leukaemia (APL) treatment If you do go into remission, but the cancer comes back, this is known as a relapse. This is especially rare if you were treated with ATRA and arsenic trioxide.
Can APML spread?
Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML), a form of blood cancer. APL is an aggressive cancer, meaning it can grow and spread rapidly.
How is APML diagnosed?
APML is diagnosed by examining samples of your blood and bone marrow.
- Full blood count. The first step in diagnosis is a simple blood test called a full blood count (FBC) or complete blood count (CBC). …
- Bone marrow examination. …
- Further testing. …
- Other tests.
How does PML-Rara cause APL?
In APL, PML-RARA alters the nuclear structure of NBs, leading to their disruption into nuclear microspeckles [17]. This is likely due to the lack of the SUMO-binding motif within the PML moiety of the chimeric protein [14], and is a diagnostic hallmark of APL [17].
How do you treat Apml?
In most people with APML, treatment with ATRA and arsenic trioxide leads to a remission. Some people with high white cell counts may also need intravenous chemotherapy. Samples of your bone marrow may be tested for a genetic change known as PML-RARA. This can help doctors work out whether you are in remission.
What is FLT3 mutation?
FLT3 is a gene change, or mutation, in leukemia (blood cancer) cells. It’s the most common genetic change in acute myeloid leukemia (AML), a type of leukemia that starts in the bone marrow and often moves into the blood. The FLT3 gene contains instructions for a protein called FLT3, which helps white blood cells grow.
What is survival rate of APML?
Unlike most leukemias, acute promyelocytic leukemia (APL) has a very good prognosis, with long-term survival rates up to 90% following treatment.
Can APL be cured as of 2021?
Because of advances in diagnostic techniques and modern treatments, APL is now considered the most curable subtype of AML in adults, with complete remission rates of 90 percent following treatment and cure rates of approximately 80 percent reported in clinical trials.
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