Acute promyelocytic leukemia: Description, Causes and Risk Factors:Abbreviation: APL.Acute promyelocytic leukemia is medically defined as an abnormal or irregular accumulation of granulocytes that have not been properly formed, a condition caused by chromosomes that end up in the wrong place within certain receptor genes.Acute promyelocytic leukemia is of rare disease and comprises approx. 20% of all patients with acute myeloid leukemia. A higher incidence rate is observed in Italy as well as in Northern, Central, and Southern America. The mean age lies between 40 and 50 years. Children, Men & Women are almost equally affected.Acute promyelocytic leukemia is characterized by proliferating abnormal promyelocytes. Most patients with this disorder present with a hemorrhagic diathesis usually associated with disseminated intravascular coagulation (DIC). It is often difficult to successfully induce remission because of DIC-associated hemorrhagic complications, particularly cerebral hemorrhage. Baker and co-workers were the first to report more than 20 years ago the successful use of heparin in the treatment of DIC in a patient with APL. Eight years later Gralnick and associates confirmed the beneficial effects of using heparin in three patients with APL. Since that time no prospective randomized study has been carried out, and recently some investigators have questioned the efficacy of using heparin in treating this disorder. There is, therefore, still some controversy regarding the ideal supportive measures necessary for inducing remission in APL.Acute promyelocytic leukemia is distinctively biologic and clinical features that is now highly curable. Most patients are young, present with leukopenia, and exhibit a life-threatening coagulopathy, which is the most notorious manifestation of the disease. The cells from almost all patients have a balanced reciprocal translocation between chromosomes 15 and 17, which generates a fusion transcript joining the PML (promyelocyte) and RAR-? (retinoic acid receptor-?) genes. Leukemic promyelocytes have the unique ability to undergo differentiation with exposure to retinoic acid and both differentiation and apoptosis with exposure to arsenic trioxide (ATO). The disease is relatively rare in adults, accounting for only 10% to 15% of the approximately 13 400 adults diagnosed with AML in the United States each year. Although the incidence of APL among children with AML is similar in some series, a higher overall percentage of APL in children with AML compared with adults has been reported in others. In children, the disease is often associated with a high white blood cell count (WBC > 10 000/?L), the microgranular variant (M3V), and more all-trans retinoic acid (ATRA)-related toxicities, particularly pseudotumor cerebri.Although most patients with APL will have DIC (disseminated intravascular coagulation), most can be successfully managed with the aggressive use of heparin plus platelet and cryoprecipitate transfusions. The initial labeling index may help predict which patients are most likely to achieve a complete remission. Those patients who have a complete remission can be expected to have a survival comparable to responders with other AML subtypes. Finally, an anthracycline or amsacrine should be included as part of the induction chemotherapy regimen. Because marrow hypoplasia is not essential for complete remission, careful review is recommended before administering second or further courses of induction therapy.Symptoms:Symptoms of APL are determined by age, overall health condition, and severity of the condition. Some of the most common include a general sense of fatigue, possible weight loss, fever and shortness of breath. Some individuals bruise easily and even develop what is known as petechiae, or tiny points of blood evident beneath the surface of the skin. Some people also experience anemia.Diagnosis:Diagnoses include the following tests:Complete physical examination (with special attention to bleeding tendency, anemic symptoms, & infections).
Complete blood cell count, including leukocyte count with differential cell counts.
Fluorescent in situ hybridization [FISH].
Additional diagnostic procedures:General health condition (ECOG/WHO Score).
Evaluation of comorbidities.
Clinical chemistry, urine analysis.
Hepatitis and HIV serology.
Pregnancy test (if applicable).
Treatment:While there is no 100% percent cure for any form of leukemia, some individuals benefit from ATRA (All-trans retinoic acid), which is a product of trans-retinoic acid, which in turn comes from vitamin A. This treatment causes poorly formed or immature promyelocytes to become fully formed or mature granulocytes, which are essential for proper growth, development and function of cells.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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