부산대학교 도서관

Introduction: Myelodysplasia/ myeloproliferative with ring sideroblasts and thrombocytosis (MDS/MPNRST) presents with anemia and platelets greater than ≥450,000 /mm3, >15% RS, or >5% with the SF3B1 mutation in the absence of the 5q deletion and blasts 1000×109/L) and risk of bleeding with antiplatelet therapy. Currently, there are no general guidelines for the treatment of this pathology, the use of hydroxyurea, alpha interferon, anagrelide and busulfan has been described. Anemia is treated with red blood cell transfusion or erythropoietin. Median survival was around 70 months for MDS/MPNRST and 21.8 months for MDS/MPNU. The AZAVIT+ABCDEF protocol, linked to the Plataforma Brasil CAAE: 53015421.0.0000.5463, consists of azacytidine associated with high doses of vitamins (B1, C and D) with erythropoietin and filgrastim, started after signing the consent form. Goal: Report a clinical case of a patient in a reference service. Method: data collection in HSPE's digital system. Clinical case: Male, 73 years old, with asthenia, weight loss of 15 kg, night sweats and recent herpes zoster. Hb: 7.1 g/dL, leukocytes: 4680/mm3, platelets: 819,000/mm3, Myelogram: Dysplastic alterations in the 3 lineages with 15.6% of blasts. Medullary iron with 25% ringed sideroblasts, positive blast immunophenotyping for: CD13, CD34, CD117, CD45, HLA-DR, CD38, CD105, partial CD7 and Bone marrow biopsy: hypercellular with dysplastic alterations in the 3 lineages with increased shapes precursors, moderate dysmegakaryocytopoiesis and dense reticulin tissue. Karyotype: 46, XY. Initially classified as (MDS/MPNRST) and reclassified to MDS/MPNU due to having 15.6% blasts in the bone marrow. The patient received 8 cycles of the protocol. Initially, maintenance of anemia with an increase in platelets, reaching 1,266,000/mm3. After the 2nd cycle, there was a drop in platelets and normalization of hemoglobin. Currently: Hb: 12.2 g/dL, leukocytes: 3630/mm3, platelets: 162,000/mm3 in July/23. Myelogram and immunophenotyping: hypercellular with dysplastic alterations and without blasts. The observed toxicity was mild joint pain. Discussion: We believe that the protocol can promote differentiation by increasing the transcription of factors related to differentiation, improving aerobic metabolism and promoting immunomodulation. Conclusion: The hematological response was important with an improvement in the patient's quality of life and possibly in the gain of survival. the AZAVIT-ABCDEF protocol should be analyzed in a prospective cohort in future studies.