Análise da terapêutica com agentes hipometilantes sobre a sobrevida em portadores de síndrome mielodisplásica, correlacionando com fatores clínicos, hematológicos, citopatogenéticos e índices prognósticos

Abstract

Therapy with hypomethylating agents, Azacitidine and Decitabine, has emerged relatively recently as an alternative treatment for myelodysplastic syndrome. An unknown number of patients used these drugs in follow - up at specialized hematology services in Fortaleza - Ceará, both at the University Hospital of the Federal University of Ceará and at the General Hospital of Fortaleza. We attempted to identify all the patients who received Azacitidine and Decitabine for the treatment of myelodysplastic syndrome in both hematology services until February 28, 2018, to define their survival and to analyze the epidemiological, hematological, and comorbidities factors and prognostic scores that would be able to influence it. From this, through an analytical-descriptive retrospective study, the medical records of all patients with myelodysplastic syndrome who had received at least one dose of one of the hypomethylating agents in both specialized hematology services were reviewed. A total sample of 52 patients was identified, mean age was 69.7 years, male predominance was 51.9%. Azacitidine users accounted for 59.6% of the total, while Decitabine alone was used for a total of 26.9% and a portion of 12.5% of patients used sequenced both hypomethylating agents. Myelodysplastic Syndrome - comorbidity index presented 80.8% of the patients classified as low risk and the age - adjusted Charlson comorbidities index had a score of three or less for 57.7% of the total sample. An unfavorable karyotype characterized 55.3% of patients who had this test available. The prognostic indices Revised - International Prognostic Scoring System and World Health Organization Classification - based Prognostic Scoring System presented high risk categorization for 71.1% and 84.2% of the available sample, respectively. Hemoglobin greater than or equal to 8 g / dL at the time of initiation of hypomethylation therapy was the only statistically significant variable associated with the occurrence of a therapeutic response to Azacitidine or Decitabine. Clinical outcomes of success or failure of hypomethylating agents were significantly influenced by the occurrence of hematologic improvement in the erythrocyte and platelet sectors. A majority of 57.7% of the sample received altogether only six therapeutic cycles of hypomethylants. The median events free survival time obtained by total patients was 18.9 months at diagnosis of myelodysplastic syndrome and 10.7 months at the time of initiation of treatment. It was concluded that presenting a subtype of myelodysplastic syndrome without excess blasts and receiving a minimum of three therapeutic cycles of one of the hypomethylating agents were the only variables that were statistically significantly associated with the events free survival established for the time of diagnosis of the syndrome myelodysplastic. Platelet count of at least 50,000 / mm3, the absence of transfusional dependence of platelets, therapeutic response in peripheral blood parameters in the erythrocyte and platelet count, favorable therapeutic response to Azacitidine or Decitabine and treatment with at least three cycles of hypomethylating agents were the only variables that presented a statistically significant association with the event-free survival established for the moment of initiation of the hypomethylating treatment.