[From the molecular model to the impact on prognosis: an overview on acute promyelocytic leukemia].
Acute promyelocytic leukemia (APL) is a model of clinical applicability of the knowledge of molecular physiopathology. It is characterized by recurrent genetic involvement of the retinoic acid alpha receptor. The consequence is a protein with low sensibility to its ligand and a myeloid maturation arrest. However, higher doses of all-trans-retinoic acid (ATRA) are able to supersede this deficiency and this is the mainstay of APL treatment leading to over 80% disease free survival, when adequately treated. Epidemiologically, it differs from other acute myeloid leukemia due to a higher incidence in young adults and in countries of "Latin" colonization. Differing from excellent results observed in developed countries, APL mortality in Brazil is still high, despite the wide availability of drugs.
Jácomo
Rafael Henriques
RH
Laboratório de Hematologia, Hospital das Clínicas, Faculdade de Medicina de Ribeirão Preto, SP, Brazil.
Figueiredo-Pontes
Lorena Lobo de
LL
Rego
Eduardo Magalhães
EM
D000970:Antineoplastic Agents / Q000627:therapeutic use; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics*; D011379:Prognosis; D014212:Tretinoin / Q000627:therapeutic use
D000970:Antineoplastic Agents; D014212:Tretinoin