What causes APL?

What causes APL?

APL is caused by a chromosomal translocation (rearrangement of material) that occurs in some of the body’s cells during a person’s lifetime (a somatic mutation ). The translocation involves the fusion of two genes : the PML gene on chromosome 15 and the RARA gene on chromosome 17.

What is APL leukemia?

Acute promyelocytic leukemia (APL) is an aggressive type of acute myeloid leukemia (AML). It happens when there are too many of the blood-forming cells called promyelocytes in the blood and bone marrow.

Can APL be cured?

Acute promyelocytic leukemia (APL) has become a curable disease by all-trans retinoic acid (ATRA)-based induction therapy followed by two or three courses of consolidation chemotherapy. Currently around 90% of newly diagnosed patients with APL achieve complete remission (CR) and over 70% of patients are curable.

Can APL be cured as of 2021?

ATRA and ATO have greatly modified the prognosis of APL. APL is now considered a curable disease.

When should you suspect APL?

Thus, in standard-risk APL, hydroxyurea should be initiated if the WBC count rises to >10 109/L, and in high-risk disease, anthracycline chemotherapy during induction should be considered.

How treatable is APL leukemia?

Because of advances in diagnostic techniques and modern treatments, APL is today considered to be the most curable subtype of acute myeloid leukemia in adults, with complete remission rates of 90 percent and cure rates of approximately 80 percent and even higher among low-risk patients.

What diagnosis is APL?

Acute promyelocytic leukemia (APL) is a blood cancer characterized by a marked increase in a type of white blood cells known as promyelocytes, a type of immature white blood cell. It develops in about 600 to 800 individuals each year in the United States, most often in adults around the age of 40.

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Is APL inherited?

APL is not inherited . The condition arises from a translocation in some of the body’s cells ( somatic cells ) that occurs after conception. This is referred to as a somatic mutation . Somatic mutations may affect the individual by causing cancers or other diseases, but they are not passed on to offspring.

How long does APL treatment take?

The most common options for maintenance therapy are ATRA alone, or ATRA along with chemo (6-mercaptopurine (6-MP) and/or methotrexate). Maintenance therapy is typically given for about a year.

Which leukemia is treated with vitamin A?

ATRA is a form of vitamin A that is typically part of the initial (induction) treatment of APL. It is given either along with chemo, or along with arsenic trioxide for the initial treatment of APL.

What causes differentiation syndrome?

Differentiation syndrome (DS; originally called retinoic acid syndrome) is a potentially fatal complication of treatment of acute promyelocytic leukemia (APL) with all-trans retinoic acid and/or arsenic trioxide, treatment of acute myeloid leukemia (AML) with inhibitors of isocitrate dehydrogenase (IDH; eg, IDH2 …

Can APL leukemia come back?

The prognosis of acute promyelocytic leukemia (APL) has been improved by the combination of all-trans retinoic acid (ATRA) with chemotherapy. Nonetheless, relapse occurs in a certain proportion of patients, mostly within three to four years after treatment.

How often does APL relapse?

Despite this, relapse occurs in 5 to 10 percent of patients with APL and in 20 to 30 percent of those with high-risk APL (ie, presentation with a white blood cell count >10,000/microL and a platelet count <40,000/microL). Some of these relapses have occurred after more than five years in remission.

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Is APL an emergency?

Acute promyelocytic leukemia (APL) represents a medical emergency with a high rate of early mortality. As a consequence, as soon as the diagnosis is suspected based upon cytologic criteria, it is necessary to start all- trans retinoic acid (ATRA) treatment without delay.

What is the most curable leukemia?

While it is similar in many ways to the other subtypes, APL is distinctive and has a very specific treatment regime. Treatment outcomes for APL are very good, and it is considered the most curable type of leukemia.

What chromosome is affected by leukemia?

Chronic myeloid leukemia is caused by a rearrangement (translocation ) of genetic material between chromosome 9 and chromosome 22. This translocation, written as t(9;22), fuses part of the ABL1 gene from chromosome 9 with part of the BCR gene from chromosome 22, creating an abnormal fusion gene called BCR-ABL1.

Is APL leukemia rare?

APL is one of the many types of leukemia. It is a relatively rare type of AML, as it only makes up about 7 to 8 percent of adult AML cases.

What is the prognosis of APL?

The prognosis of APL is very poor without treatment (median survival~1 month). However, with modern treatment, survival has improved greatly and most patients achieve complete remission and remain so.

How do you test for acute promyelocytic leukemia?

APML is diagnosed by examining samples of your blood and bone marrow.

  1. Full blood count. The first step in diagnosis is a simple blood test called a full blood count (FBC) or complete blood count (CBC). …
  2. Bone marrow examination. …
  3. Further testing. …
  4. Other tests.
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How do you treat high risk APL?

Currently, there are 2 potential treatment options for high-risk patients, that is, ATRA plus ATO with the addition of some cytoreductive chemotherapy and ATRA plus chemotherapy, because neither has yet been shown to be superior in randomized studies.