Background: Polycythemia Vera (PV) and Essential Thrombocythemia (ET) are long term outcome myeloproliferative neoplasm (MPN); however, they could evolve to secondary myelofibrosis-MF or acute leukemia-AL. Prognostic value of bone marrow (BM) fibrosis grading in PV and TE patients is still debated. Aims and Methods: We retrospectively analyzed a cohort of 579 PV (n=180) and ET (n=399) patients, diagnosed between 1990 and 2013 in Turin and Bologna, and examined the prevalence and prognostic relevance of BM reticulin fibrosis. Eligibility criteria included the availability of BM samples at diagnosis. BM biopsy sample were reviewed by local pathologist and fiber scoring was performed according to a 3-graded system. Patients with grade 2 or 3 fibrosis were excluded. We evaluated overall survival (OS) using Kaplan Meyer method and HR were estimated with the Cox Model. Cumulative incidence (CI) of MF and AL evolution were estimated considering death from any cause. Results:115 (63%) grade 0 and 65 (36%) grade 1 fibrosis and 291 (72%) grade 0 and 108 (27%) grade 1 among PV and ET cases, respectively (p= 0.028) We analyzed effect on clinical outcome separately. PV: at a median follow up of 110 months (IQR:70- 170), 5 and 10-years OS were 96% and 87%, respectively. Stratified by fibrosis degree the 5 and 10-years OS were 98% vs 90% and 92% vs 82% for grade 0 and grade 1 (p 0.076), respectively. CI of MF evolution at 5 and 10-years was 2,8% and 7,2% vs 3,8% and 18,7% for grade 0 and grade 1 (p 0.123). CI of AL evolution at 10- year was 4,2% for both grade whereas at 15-years was 4,2% vs 19% for grade 0 and 1. ET: at a median follow up of 75 months (IQR:39-120), 5 and 10-years OS were 98% and 90%, respectively. The 5 and 10-years OS were 98% and 90% for grade 0 vs 97% and 89% for grade 1 (p 0.358), respectively, when stratified by fibrosis degree. Mutation status was analyzed in 379 TE patients. CI of MF evolution was at 5-years 0,5% and 9% and at 10- years 6,2% and 18% for grade 0 and 1 (p 0.0001). CI of AL evolution at 5 and 10-years was 0% for grade 0 and 13% and 7,3% for grade 1(p 0.096). Grade 0 showed a higher cumulative risk of AL evolution at 15- years (6,9% vs 10% for grade 0 and 1) (p=0.096). Conclusions: In ET patients grade 1 BM fibrosis seems to correlate with a higher cumulative risk of MF and AL evolution, whereas in PV patients seems to correlate to a trend of higher mortality, even if not statistically significantly.
EFFECT ON CLINICAL OUTCOME OF BONE MARROW RETICULIN FIBROSIS IN 579 PATIENTS WITH POLYCYTHEMIA VERA AND ESSENTIAL THROMBOCYTHEMIA
RIERA, Ludovica;PICH, Achille;
2017-01-01
Abstract
Background: Polycythemia Vera (PV) and Essential Thrombocythemia (ET) are long term outcome myeloproliferative neoplasm (MPN); however, they could evolve to secondary myelofibrosis-MF or acute leukemia-AL. Prognostic value of bone marrow (BM) fibrosis grading in PV and TE patients is still debated. Aims and Methods: We retrospectively analyzed a cohort of 579 PV (n=180) and ET (n=399) patients, diagnosed between 1990 and 2013 in Turin and Bologna, and examined the prevalence and prognostic relevance of BM reticulin fibrosis. Eligibility criteria included the availability of BM samples at diagnosis. BM biopsy sample were reviewed by local pathologist and fiber scoring was performed according to a 3-graded system. Patients with grade 2 or 3 fibrosis were excluded. We evaluated overall survival (OS) using Kaplan Meyer method and HR were estimated with the Cox Model. Cumulative incidence (CI) of MF and AL evolution were estimated considering death from any cause. Results:115 (63%) grade 0 and 65 (36%) grade 1 fibrosis and 291 (72%) grade 0 and 108 (27%) grade 1 among PV and ET cases, respectively (p= 0.028) We analyzed effect on clinical outcome separately. PV: at a median follow up of 110 months (IQR:70- 170), 5 and 10-years OS were 96% and 87%, respectively. Stratified by fibrosis degree the 5 and 10-years OS were 98% vs 90% and 92% vs 82% for grade 0 and grade 1 (p 0.076), respectively. CI of MF evolution at 5 and 10-years was 2,8% and 7,2% vs 3,8% and 18,7% for grade 0 and grade 1 (p 0.123). CI of AL evolution at 10- year was 4,2% for both grade whereas at 15-years was 4,2% vs 19% for grade 0 and 1. ET: at a median follow up of 75 months (IQR:39-120), 5 and 10-years OS were 98% and 90%, respectively. The 5 and 10-years OS were 98% and 90% for grade 0 vs 97% and 89% for grade 1 (p 0.358), respectively, when stratified by fibrosis degree. Mutation status was analyzed in 379 TE patients. CI of MF evolution was at 5-years 0,5% and 9% and at 10- years 6,2% and 18% for grade 0 and 1 (p 0.0001). CI of AL evolution at 5 and 10-years was 0% for grade 0 and 13% and 7,3% for grade 1(p 0.096). Grade 0 showed a higher cumulative risk of AL evolution at 15- years (6,9% vs 10% for grade 0 and 1) (p=0.096). Conclusions: In ET patients grade 1 BM fibrosis seems to correlate with a higher cumulative risk of MF and AL evolution, whereas in PV patients seems to correlate to a trend of higher mortality, even if not statistically significantly.
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