Leucemia promielocítica aguda: do histórico ao tratamento

Abstract

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia characterized by hemorrhagic events that can cause the patient's death, if not resolved quickly, thus requiring rapid and accurate diagnosis so that treatment can be started early. Considering the role of the biomedical professional in contributing to the diagnosis of pathologies, the main of this work was to perform a bibliographic survey on APL, including its definition, history, clinical presentation, pathophysiology, epidemiology, diagnosis and treatment, in order to gather information about this neoplasm. To this end, searches were conducted in PubMed, SciELO, Google Scholar and Science Direct databases, in the period between May and December 2022, using search keys that combined “acute promyelocytic leukemia” and a second keyword, such as “bleeding”, “trans-retinoic acid”, “coagulopathies”, “acute myeloid leukemia”, “diagnosis”, “prognosis”, “translocation”, “incidence”, “hemostatic diseases”, as well as their English counterparts and abbreviations “LPA”, “APL”, “LMA”, “AML” and “ATRA”. The results show that acute promyelocytic leukemia has as its pathophysiology the occurrence of chromosomal alterations, most frequently the reciprocal chromosomal translocation t(15;17)(q22;q21), leading to the formation of the PML/RARα fusion protein, which causes the blockage of granulocyte maturation at the promyelocyte stage. Patients are affected by a state of severe coagulopathy that can have an early fatal outcome if not resolved quickly and whose main clinical complications are hemorrhagic and thrombotic conditions. Fortunately, the prognosis of this disease has undergone a conversion from "most malignant form of acute leukemia" to "highly curable" over the years, thanks to the implementation of therapies that combine retinoic acid derivatives and arsenic trioxide to induce maturation of the abnormal promyelocytes and degradation of the fusion oncoprotein. The diagnosis is made by a set of tests taht range from the clinical presentation to laboratory tests with the analysis of bone marrow aspirate by genetic-molecular techniques, being the polymerase chain reaction followed by reverse transcriptase the gold standard for the diagnosis and monitoring of the disease. From the results obtained, it is possible to conclude that APL is a pathology whose history includes several scientific advances, which have contributed positively to the change in the prognosis of the disease. In addition, the importance of a trained professional for the recognition of laboratory abnormalities and the consequent direction of the diagnosis is reinforced, contributing to the early outline of bleeding events and consequent death.