Yazarlar : Lussana F, Rambaldi A, Finazzi MC

Yayın : Haematologica

Yayın Yılı : 2014

Pubmed Linki : http://www.ncbi.nlm.nih.gov/pubmed/24389309

Konu : Kemik İliği Nakli

Literatür İçeriği :  The clinical course of polycythemia vera and essential thrombocythemia is potentially associated with long-term severe complications, such as evolution to myelofibrosis or acute myeloid leukemia. Allogeneic stem cell transplantation is currently the only potentially curative treatment for advanced polycythemia vera or essential thrombocythemia. We analyzed 250 consecutive patients with an initial diagnosis of polycythemia vera (n=120) or essential thrombocythemia (n=130), who underwent transplantation due to progression to myelofibrosis (n=193) or acute myeloid leukemia (n=57) and reported to European Group for Blood and Marrow Transplantation registry between 1994 and 2010. The median age was 56 years (range 22-75) and 52% of patients had an interval from diagnosis to transplant of 10 years or more. With a median follow-up from transplantation of 13 months, 3 years overall survival and relapse incidence were 55% and 32%, respectively. In the univariate analysis, the main parameters that negatively affected post-transplantation outcomes were older age (>55 years), a diagnosis at transplant of acute myeloid leukemia and the use of an unrelated donor. The 3 years cumulative incidence of non-relapse mortality was 28%, significantly higher in older patients (>55years, 35% vs. 20%, p=0.032), in unrelated compared to related donor (34% vs. 18%, p=0.034) and in diagnosis of acute myeloid leukemia compared to myelofibrosis (29% vs. 27%, p=0.045). This large retrospective study confirms that transplantation is potentially curative for end-stage polycythemia vera /essential thrombocythemia patients progressing to myelofibrosis or acute myeloid leukemia. Relapse and non-relapse mortality remain unsolved problems for which innovative treatment approaches need to be assessed.

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