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Conference Paper: Clinicopathologic features and outcome of Chinese patients with myelofibrosis

TitleClinicopathologic features and outcome of Chinese patients with myelofibrosis
Authors
Issue Date2014
PublisherAmerican Society of Hematology. The Journal's web site is located at http://bloodjournal.hematologylibrary.org/
Citation
The 56th Annual Meeting and Exposition of the American Society of Hematology (ASH 2014), San Francisco, CA., 6-9 December 2014. In Blood, 2014, v. 124 n. 21 How to Cite?
AbstractINTRODUCTION: Myelofibrosis (MF) is a myeloproliferative neoplasm (MPN) comprising primary myelofibrosis (PMF) and myelofibrosis developing from either polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). METHODS: Two hundred and sixty-seven consecutive patients with MF (PMF, N=206); post-PV MF, N=26; post-ET MF, N=35) diagnosed between January 1988 and December 2013 at seven regional hospitals in Hong Kong were prospectively followed up. Data on the clinicopathologic features and treatment outcome were collected. RESULTS: The median duration of follow-up was 45 (1 – 247) months. The median overall survival (OS) was 65 months (95% confidence interval, CI: 56.3–73.7). The 5-year and 10-year OS were 52.2% and 26.7% respectively. On univariate analysis, factors associated with an inferior OS included age > 65 years (hazard ratio ,HR, =1.81; 95% CI:1.33–2.48; P<0.001), the presence of constitutional symptoms (HR=1.52; 95% CI:1.11–2.08; P=0.009), hemoglobin < 10 g/dL (HR=1.71; 95% CI:1.25–2.35; P=0.001), circulating blasts ≥ 1% (HR=1.55; 95% CI:1.13–2.16; P=0.007), platelet < 100 x 109/L (HR=2.51; 95% CI:1.79–3.53; P<0.001), high risk IPSS (HR=3.09; 95% CI: 1.78–5.37; P<0.001), intermediate-2 risk DIPSS (HR=2.37; 95% CI: 1.38–4.06; P=0.002), high risk DIPSS (HR=2.92; 95% CI:1.51–5.64; P=0.001), transfusion dependence within the first year of diagnosis (HR=2.61; 95% CI:1.92–3.55; P<0.001), transfusion dependence after the first year of diagnosis (HR=2.42; 95% CI:1.61–3.54; P<0.001), palpable hepatomegaly at diagnosis (HR=1.44; 95% CI:1.06–1.96; P=0.02) and secondary AML (HR=1.75; 95% CI:1.24–2.47; P=0.001). On multivariate analysis, post-PV MF (P=0.03), platelet < 100 × 109/L (P=0.001), high risk IPSS (P=0.009), transfusion dependence within the first year (P=0.001), transfusion dependence after the first year (P=0.02), and transformation to secondary acute myeloid leukemia (AML) (P=0.007) were independent risks associated with inferior OS. On univariate analysis, factors associated with increased risk of secondary AML include age ≤ 55 years (odds ratio [OR] = 2.61; 95% CI:1.33–5.12; P=0.005), circulating blasts ≥ 1% (OR=2.24; 95% CI:1.18–4.26; P=0.01), transfusion dependence within the first year (OR=5.57; 95%:2.16–14.88; P<0.001), transfusion dependence after the first year (OR=5.57; 95% CI:2.16–14.88; P<0.001), hepatomegaly (OR=4.05; 95% CI:2.03–8.10), splenomegaly (OR=3.71; 95% CI:1.09–9.26; P=0.04), abnormal karyotypes (OR=3.3; 95% CI:1.14–9.56; P=0.02), and the presence of unfavourable karyotypes (OR=4.9; 95% CI: 1.22–19.96). On multivariate analysis, transfusion dependence within the first year of diagnosis (P=0.04), transfusion dependence after the first year of diagnosis (P=0.003) and hepatomegaly (P=0.006) were independent risks associated with secondary AML. The 5-year and 10-year risks of leukemic transformation were 17.1% and 29.7 respectively. Factors associated with inferior leukemia-free survival (LFS) on univariate analysis included post-ET MF (HR=2.15; 95% CI: 1.03–4.50; P=0.04), presence of constitutional symptoms (HR=1.85; 95% CI:1.04–3.31; P=0.04), circulating blasts ≥ 1% (HR=2.89; 95% CI:1.62–5.16; P<0.001), platelet count < 100 x 109/L (HR=2.56; 95% CI: 1.35–4.84; P=0.004), transfusion dependence within the first year of diagnosis (HR=3.05; 95% CI: 1.70–5.50; P<0.001), transfusion dependence after the first year of diagnosis (HR=6.49; 95% CI: 2.33–18.10; P<0.001), hepatomegaly (HR=3.21; 95% CI:1.69–6.08; P<0.001) and unfavorable karyotype (HR=3.01; 95% CI:1.08–8.35; P=0.03). On multivariate analysis, male gender (P=0.05), presence of constitutional symptoms (P=0.04) and unfavorable karyotypes (P=0.01) were independent risks associated with inferior LFS. Eighteen patients underwent allogeneic haematopoietic stem cell transplantation (HSCT) (matched sibling, N=14; matched-unrelated, N=4), with 15 patients achieving complete remission. Seven patients relapsed with subsequent progression to secondary AML. Acute and chronic graft-versus-host disease occurred in seven (39.9%) and six (33.3%) patients respectively. Transplant-related mortality occurred in three patients. The 5-year and 10-year OS following HSCT was 51.5%. CONCLUSION: Findings of this study complement current prognostic models in guiding treatment decisions at diagnosis and during the course of MF. © 2014 by The American Society of Hematology
DescriptionThis journal issue contain 2014 ASH Annual Meeting Abstracts
Persistent Identifierhttp://hdl.handle.net/10722/207342
ISSN
2015 Impact Factor: 11.841
2015 SCImago Journal Rankings: 6.395

 

DC FieldValueLanguage
dc.contributor.authorSingh, GHHen_US
dc.contributor.authorHwang, YYGen_US
dc.contributor.authorChan, TSYen_US
dc.contributor.authorChan, CCen_US
dc.contributor.authorChan, CHNen_US
dc.contributor.authorLiu, HSYen_US
dc.contributor.authorMak, Ven_US
dc.contributor.authorLin, SYen_US
dc.contributor.authorLau, CKen_US
dc.contributor.authorLeung, AYHen_US
dc.contributor.authorLie, AKWen_US
dc.contributor.authorKwong, YLen_US
dc.date.accessioned2014-12-19T10:54:26Z-
dc.date.available2014-12-19T10:54:26Z-
dc.date.issued2014en_US
dc.identifier.citationThe 56th Annual Meeting and Exposition of the American Society of Hematology (ASH 2014), San Francisco, CA., 6-9 December 2014. In Blood, 2014, v. 124 n. 21en_US
dc.identifier.issn0006-4971-
dc.identifier.urihttp://hdl.handle.net/10722/207342-
dc.descriptionThis journal issue contain 2014 ASH Annual Meeting Abstracts-
dc.description.abstractINTRODUCTION: Myelofibrosis (MF) is a myeloproliferative neoplasm (MPN) comprising primary myelofibrosis (PMF) and myelofibrosis developing from either polycythemia vera (post-PV MF) or essential thrombocythemia (post-ET MF). METHODS: Two hundred and sixty-seven consecutive patients with MF (PMF, N=206); post-PV MF, N=26; post-ET MF, N=35) diagnosed between January 1988 and December 2013 at seven regional hospitals in Hong Kong were prospectively followed up. Data on the clinicopathologic features and treatment outcome were collected. RESULTS: The median duration of follow-up was 45 (1 – 247) months. The median overall survival (OS) was 65 months (95% confidence interval, CI: 56.3–73.7). The 5-year and 10-year OS were 52.2% and 26.7% respectively. On univariate analysis, factors associated with an inferior OS included age > 65 years (hazard ratio ,HR, =1.81; 95% CI:1.33–2.48; P<0.001), the presence of constitutional symptoms (HR=1.52; 95% CI:1.11–2.08; P=0.009), hemoglobin < 10 g/dL (HR=1.71; 95% CI:1.25–2.35; P=0.001), circulating blasts ≥ 1% (HR=1.55; 95% CI:1.13–2.16; P=0.007), platelet < 100 x 109/L (HR=2.51; 95% CI:1.79–3.53; P<0.001), high risk IPSS (HR=3.09; 95% CI: 1.78–5.37; P<0.001), intermediate-2 risk DIPSS (HR=2.37; 95% CI: 1.38–4.06; P=0.002), high risk DIPSS (HR=2.92; 95% CI:1.51–5.64; P=0.001), transfusion dependence within the first year of diagnosis (HR=2.61; 95% CI:1.92–3.55; P<0.001), transfusion dependence after the first year of diagnosis (HR=2.42; 95% CI:1.61–3.54; P<0.001), palpable hepatomegaly at diagnosis (HR=1.44; 95% CI:1.06–1.96; P=0.02) and secondary AML (HR=1.75; 95% CI:1.24–2.47; P=0.001). On multivariate analysis, post-PV MF (P=0.03), platelet < 100 × 109/L (P=0.001), high risk IPSS (P=0.009), transfusion dependence within the first year (P=0.001), transfusion dependence after the first year (P=0.02), and transformation to secondary acute myeloid leukemia (AML) (P=0.007) were independent risks associated with inferior OS. On univariate analysis, factors associated with increased risk of secondary AML include age ≤ 55 years (odds ratio [OR] = 2.61; 95% CI:1.33–5.12; P=0.005), circulating blasts ≥ 1% (OR=2.24; 95% CI:1.18–4.26; P=0.01), transfusion dependence within the first year (OR=5.57; 95%:2.16–14.88; P<0.001), transfusion dependence after the first year (OR=5.57; 95% CI:2.16–14.88; P<0.001), hepatomegaly (OR=4.05; 95% CI:2.03–8.10), splenomegaly (OR=3.71; 95% CI:1.09–9.26; P=0.04), abnormal karyotypes (OR=3.3; 95% CI:1.14–9.56; P=0.02), and the presence of unfavourable karyotypes (OR=4.9; 95% CI: 1.22–19.96). On multivariate analysis, transfusion dependence within the first year of diagnosis (P=0.04), transfusion dependence after the first year of diagnosis (P=0.003) and hepatomegaly (P=0.006) were independent risks associated with secondary AML. The 5-year and 10-year risks of leukemic transformation were 17.1% and 29.7 respectively. Factors associated with inferior leukemia-free survival (LFS) on univariate analysis included post-ET MF (HR=2.15; 95% CI: 1.03–4.50; P=0.04), presence of constitutional symptoms (HR=1.85; 95% CI:1.04–3.31; P=0.04), circulating blasts ≥ 1% (HR=2.89; 95% CI:1.62–5.16; P<0.001), platelet count < 100 x 109/L (HR=2.56; 95% CI: 1.35–4.84; P=0.004), transfusion dependence within the first year of diagnosis (HR=3.05; 95% CI: 1.70–5.50; P<0.001), transfusion dependence after the first year of diagnosis (HR=6.49; 95% CI: 2.33–18.10; P<0.001), hepatomegaly (HR=3.21; 95% CI:1.69–6.08; P<0.001) and unfavorable karyotype (HR=3.01; 95% CI:1.08–8.35; P=0.03). On multivariate analysis, male gender (P=0.05), presence of constitutional symptoms (P=0.04) and unfavorable karyotypes (P=0.01) were independent risks associated with inferior LFS. Eighteen patients underwent allogeneic haematopoietic stem cell transplantation (HSCT) (matched sibling, N=14; matched-unrelated, N=4), with 15 patients achieving complete remission. Seven patients relapsed with subsequent progression to secondary AML. Acute and chronic graft-versus-host disease occurred in seven (39.9%) and six (33.3%) patients respectively. Transplant-related mortality occurred in three patients. The 5-year and 10-year OS following HSCT was 51.5%. CONCLUSION: Findings of this study complement current prognostic models in guiding treatment decisions at diagnosis and during the course of MF. © 2014 by The American Society of Hematology-
dc.languageengen_US
dc.publisherAmerican Society of Hematology. The Journal's web site is located at http://bloodjournal.hematologylibrary.org/-
dc.relation.ispartofBlooden_US
dc.titleClinicopathologic features and outcome of Chinese patients with myelofibrosisen_US
dc.typeConference_Paperen_US
dc.identifier.emailSingh, GHH: gillhsh@hku.hken_US
dc.identifier.emailHwang, YYG: yyhwang@hku.hken_US
dc.identifier.emailLeung, AYH: ayhleung@hku.hken_US
dc.identifier.emailLie, AKW: akwlie@hku.hken_US
dc.identifier.emailKwong, YL: ylkwong@hku.hken_US
dc.identifier.authoritySingh, GHH=rp01914en_US
dc.identifier.authorityLeung, AYH=rp00265en_US
dc.identifier.authorityKwong, YL=rp00358en_US
dc.description.naturelink_to_OA_fulltext-
dc.identifier.hkuros241795en_US
dc.identifier.volume124en_US
dc.identifier.issue21en_US
dc.publisher.placeUnited States-

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