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Allogeneic Hematopoietic Stem Cell Transplantation (HSCT) is indicated for patients with intermediate or high risk primary Myelofibrosis (MF), and also in patients with Policitemia Vera or Essential Thrombocythemia who have progressed to high risk MF. The results for allogeneic HSCT with myeloablative conditioning seem to be better for patients younger than 45 years, determining lower relapse risk. However, Reduced Intensity Conditioning (RIC) for patients between 45 and 65 years-old has shown to be a promising strategy, with less mortality related to the procedure. This study aims describing a series of patients diagnosed with myelofibrosis, transplanted in the Hospital de Clínicas (HC) from the Federal University of Paraná and Hospital Nossa Senhora das Graças (HNSG) (Curitiba, Brazil).
Patients and Methods
From 1984 to 2011, fourteen patients with MF were submitted to HSCT, eleven from the HC and three from HNSG. The median of age was 42 years (10-51). There were ten males and four females. In average, five blood transfusions were done per patient (0-61). Five male patients received the graft from a female donor, and two patients received an unrelated HSCT. The median of duration of the disease was 20 months (2-150). According to Dupriez Classification, all the patients were of intermediate and high risk. Five patients had a myeloablative conditioning (Busulfan plus Cyclophosphamide: 4; Cyclophosphamide plus Total Body Irradiation:1) while nine had a RIC transplant (Fludarabine 150mg/m2 plus Melphalan 140mg/m2:6; Fludarabine plus Melphalan plus Antithymocyte Globulin:2; Fludarabine 180mg/m2 plus Bulsufan 10mg/m2 plus Antithymocyte globulin 5mg/kg:1).
Results
In the myeloablative conditioning group (n=5), all the patients presented marrow engraftment; one relapsed. Three patients presented grade II-IV acute graft versus host disease, and four developed severe chronic graft versus host disease. Four patients died, and the only survivor was 10 years-old. Median survival was 479 days. In RIC group (n=9), engraftment didn’t occur in one patient (which had splenomegaly of 18 cm at the time of transplantation), and another three relapsed. The other seven patients remained alive and free of disease, with median survival of 750 days (34-1872; p=0,000123).
Conclusion
In spite of the limited number of patients in this study, data suggest that RIC regimens improved survival in HSCT for myelofibrosis in our center, even with a slightely greater relapse risk. Except for very young patients, this strategy should be considered for further investigation.