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Acute Promyelocytic Leukemia

What is Acute Promyelocytic Leukemia?

Acute Promyelocytic Leukemia is a subtype of acute myelogenous leukemia, a cancer of the blood and bone marrow.

 

 

Acute Promyelocytic Leukemia is a subtype of acute myelogenous leukemia, a cancer of the blood and bone marrow.

 

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8.0http://www.orpha.net
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What causes acute promyelocytic leukemia? Acute promyelocytic leukemia (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. The protein produced by this fusion is referred to as PML-RARα. The PML-RARα protein functions differently than what is typically produced by the normal PML and RARA genes. As a result of the abnormal function, blood cells become "stuck" at the promyelocyte stage and they proliferate (reproduce) abnormally. Excess promyelocytes then accumulate in the bone marrow, disrupting the formation of normal white blood cells and leading to APL. Translocations involving the RARA gene and other genes have been identified in only a few cases of APL.[3] Last updated: 2/6/2012
https://leukaemialymphomaresearch.org.uk/information/leukaemia/acute-promyelocytic-leukaemia-apl/what-are-signs-and-symptoms What are the signs and symptoms of acute promyelocytic leukaemia? There are no specific symptoms of acute promyelocytic leukaemia (APL) and the condition can be confused with other common illnesses. In general APL develops very quickly and the symptoms appear over a matter of days or weeks. Common symptoms Unusual bleeding and bruising Paleness Tiredness and breathlessness Frequent and persistent infections These are caused by a lack of healthy red and white cells and platelets in the blood. Bleeding is a serious symptom of APL and needs immediate medical attention. Less common symptoms Other, less common symptoms include: Bone pain due to a build up of cancer cells in the bone marrow Swollen glands due to a build up of cancer cells in the lymph nodes Abdominal pain due to a swollen liver or spleen Some people with APL may also develop small lumps on their skin, called chloromas, but this is very uncommon. These form when leukaemia cells cluster under the skin. A very few people experience symptoms such as dizziness and bad circulation. This happens when leukaemia cells interfere with the blood supply to the central nervous system. People with APL may experience all, or just some, of these symptoms. What causes it? Publications Acute Promyelocytic Leukaemia (APL) Last updated: 02/04/2012 - See more at: https://leukaemialymphomaresearch.org.uk/information/leukaemia/acute-promyelocytic-leukaemia-apl/what-are-signs-and-symptoms#sthash.HCkTjqLb.dpuf
http://bloodjournal.hematologylibrary.org/content/113/9/1875.full#T1 3. Approach to the patient with suspected APL Although there is a general consensus on the need to confirm the diagnosis of APL at the genetic level, both differentiation and supportive therapy should be started before the results of genetic tests are available. In the majority of cases the diagnosis is suggested by the characteristic morphology of the leukemic population,17,18 however, it is also important to consider the possibility of APL should any suspicion be raised based on immunophenotypic profile or presence of severe coagulopathy; in all such cases ATRA should be commenced immediately and continued until the diagnosis is confirmed or refuted at the genetic level. 3.1. Rapid confirmation of genetic diagnosis Because the efficacy of differentiation treatment based on retinoids and/or arsenic derivatives is strictly dependent on the presence of the PML/RARA fusion in leukemia cells, genetic confirmation of this specific lesion is mandatory in all cases. Morphologic diagnosis of hypergranular (typical) APL is highly predictive of an underlying PML/RARA rearrangement, and immunophenotyping by multiparameter flow cytometry can improve the accuracy of diagnosis,19,20 particularly in patients with morphologic features evoking a microgranular (variant) subtype. However, patients with morphologic and/or immunophenotypic features suggestive of APL without the PML/RARA rearrangement, and vice versa, have been described in the literature.21–23
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http://rarediseases.info.nih.gov/gard/538/resources/more-about-this-disease#5948 How might acute promyelocytic leukemia be treated? Most cases of acute promyelocytic leukemia (APL) are treated with an anthracycline chemotherapy drug (daunorubicin or idarubicin) plus the non-chemotherapy drug, all-trans-retinoic acid (ATRA), which is a relative of vitamin A. This treatment leads to remission in 80% to 90% of patients.[4] Patients who cannot tolerate an anthracycline drug may get ATRA plus another drug called arsenic trioxide.[4] Arsenic trioxide has also proven to be an effective alternative for the 20% to 30% of patients with APL who don't respond to initial treatment or who relapse. If treatment with arsenic trioxide achieves a remission, further courses of this drug may be given. An autologous stem cell transplant may also be an option. If a second remission is not achieved, treatment options may include an allogeneic stem cell transplant or taking part in a clinical trial.[5] Additional information related to treatment of acute promyelocytic leukemia can be accessed through eMedicine. This includes detailed information related to the use of arsenic trioxide. Last updated: 2/3/2012 NOTE: ATO (arsenic trioxide) is an emerging induction treatment for APL: http://bloodjournal.hematologylibrary.org/content/113/9/1875.full
https://leukaemialymphomaresearch.org.uk/information/leukaemia/acute-promyelocytic-leukaemia-apl/prognosis Acute promyelocytic leukaemia prognosis Acute promyelocytic leukaemia (APL) is much more successfully treated now than other forms of acute myeloid leukaemia (AML). Here you will find information about the likely outcome for patients with APL. If you are a patient, you may prefer not to read this now, so do not continue. However, you may like a family member or friend to read it instead. Following treatment more than 70% of patients with APL are cured. The likelihood of being cured depends very much on age, general fitness, the subtype of APL and how far the disease had progressed when the patient was diagnosed. Younger patients who are in good general health are more likely to respond better to treatment. Last updated: 16/05/2013 - See more at: https://leukaemialymphomaresearch.org.uk/information/leukaemia/acute-promyelocytic-leukaemia-apl/prognosis#sthash.gtJyQcEk.dpuf
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Resources Supporting APL

Link to GARD web site with listings of resources for APL.

03/20/2017

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Visit sanfordresearch.org/CoRDS to enroll.

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