RISK FACTORS AND OUTCOMES OF ASIAN PATIENTS WITH MYELOPROLIFERATIVE NEOPLASMS (MPNS) IN LEUKAEMIC TRANSFORMATION
(Abstract release date: 05/19/16)
EHA Library. Kam G. 06/09/16; 134920; PB2020
Dr. Grace Kam
Contributions
Contributions
Abstract
Abstract: PB2020
Type: Publication Only
Background
Progression to acute leukaemia is a rare event in the natural history of the MPNs, namely essential thrombocythaemia (ET), polycythaemia vera (PV) and myelofibrosis. Prognosis in MPN patients with leukaemia transformation (LT) is almost universally fatal and no effective treatment is available.
Aims
As data on Asian patients with MPN in LT is lacking, we evaluated the risk factors and outcomes of Asian MPN patients with LT in this retrospective single centre analysis.
Methods
We reviewed the case records of 824 Asian patients with MPNs from our institution’s IRB approved MPN registry. Patients with LT were compared with other patients in the registry who did not have LT on follow up.
Results
Among 824 MPN patients, 30 were identified to have LT from 2002 to 2015. The median follow up was 6.2years. Median age of LT was 72years and occurred at a median of 33.2years after diagnosis. 3.9% (n=22) of ET, 0.5% (n=1) of PV and 11.5% (n=7) of myelofibrosis patients progressed to acute leukaemia (p <0.05). Time to LT was longest for ET (11years), followed by myelofibrosis (3.9years) and PV (1.5years) (p=0.018). There was no impact of JAK2, Calreticulin and MPL mutations on LT (p=0.55). Patients with LT had lower Hb at diagnosis (12.7g/dL vs. 14.8g/dL, p=0.001). A trend to higher presenting leukocyte count (15.5x 109/L vs. 12.6 x 109/L, p=0.053) and older age of diagnosis of MPN (59.5years vs. 56years, p=0.061) was seen in patients with LT (p=0.053).On log-rank test, MPN subtype (p<0.05), presenting Hb <10g/dL (p<0.05), presenting WBC ≥ 13x109/L (p=0.009), presenting platelet count ≥ 1000 x109/L (p=0.045) and age at diagnosis of MPN ≥ 60years (p=0.001) were factors for shorter time to LT. Use of hydroxyurea (p=0.17) and exposure to more than one cytoreductive agent (p=0.45) had no impact on LT. Using all co-variates with a p value ≤0.10, we developed a model using Cox proportional hazard model to determine the relative effect of these variables on LT. The model included the above variables and presence of splenomegaly at diagnosis (p=0.071). A presenting leukocyte count ≥ 13x109/L (HR 5.73, 95% Confidence Interval [CI] 1.84-17.88, p=0.03) and presenting Hb <10g/dL (HR 8.05, 95%CI 1.66-38.95, p=0.01) were independent poor prognostic factors for LT.At LT, 43.3% (n=13) had complex/ adverse cytogenetics. 30% (n=9) had clonal progression compared to karyotype analysis at diagnosis (seven did not have prior cytogenetics results). Median survival after LT was 2.5months. A diagnosis of LT after 2009 (when azacytidine became available at our institution) was not shown to affect survival after LT (p=0.73). Outcomes were not different whether LT occurred below or above the age of 60years (p=0.73). One patient (3.3%) was lost to follow up. 63.3% (n=19) received supportive treatment including palliative chemotherapy. 16.7% (n=5) received azacytidine-based chemotherapy, while 13.3% (n=4) received an AML-type regimen/ allogeneic stem cell transplant and one (3.3%) received experimental treatment. Survival for the different treatment modalities was not statistically significant (p=0.19): palliative chemotherapy/ supportive care 1.5months, azacytidine-based 3.1months, intensive chemotherapy/ allogeneic stem cell transplant 8.1months.
Conclusion
As in published literature, outcomes for Asian MPN patients in blast phase are dismal. In this study, a presenting Hb <10g/dL and presenting WBC ≥ 13x109/L were predictors of LT in MPN. Existing treatment modalities are ineffective in attaining long term remission and have not improved survival over time.
Session topic: E-poster
Keyword(s): Leukemia, Myeloproliferative disorder, Outcome
Type: Publication Only
Background
Progression to acute leukaemia is a rare event in the natural history of the MPNs, namely essential thrombocythaemia (ET), polycythaemia vera (PV) and myelofibrosis. Prognosis in MPN patients with leukaemia transformation (LT) is almost universally fatal and no effective treatment is available.
Aims
As data on Asian patients with MPN in LT is lacking, we evaluated the risk factors and outcomes of Asian MPN patients with LT in this retrospective single centre analysis.
Methods
We reviewed the case records of 824 Asian patients with MPNs from our institution’s IRB approved MPN registry. Patients with LT were compared with other patients in the registry who did not have LT on follow up.
Results
Among 824 MPN patients, 30 were identified to have LT from 2002 to 2015. The median follow up was 6.2years. Median age of LT was 72years and occurred at a median of 33.2years after diagnosis. 3.9% (n=22) of ET, 0.5% (n=1) of PV and 11.5% (n=7) of myelofibrosis patients progressed to acute leukaemia (p <0.05). Time to LT was longest for ET (11years), followed by myelofibrosis (3.9years) and PV (1.5years) (p=0.018). There was no impact of JAK2, Calreticulin and MPL mutations on LT (p=0.55). Patients with LT had lower Hb at diagnosis (12.7g/dL vs. 14.8g/dL, p=0.001). A trend to higher presenting leukocyte count (15.5x 109/L vs. 12.6 x 109/L, p=0.053) and older age of diagnosis of MPN (59.5years vs. 56years, p=0.061) was seen in patients with LT (p=0.053).On log-rank test, MPN subtype (p<0.05), presenting Hb <10g/dL (p<0.05), presenting WBC ≥ 13x109/L (p=0.009), presenting platelet count ≥ 1000 x109/L (p=0.045) and age at diagnosis of MPN ≥ 60years (p=0.001) were factors for shorter time to LT. Use of hydroxyurea (p=0.17) and exposure to more than one cytoreductive agent (p=0.45) had no impact on LT. Using all co-variates with a p value ≤0.10, we developed a model using Cox proportional hazard model to determine the relative effect of these variables on LT. The model included the above variables and presence of splenomegaly at diagnosis (p=0.071). A presenting leukocyte count ≥ 13x109/L (HR 5.73, 95% Confidence Interval [CI] 1.84-17.88, p=0.03) and presenting Hb <10g/dL (HR 8.05, 95%CI 1.66-38.95, p=0.01) were independent poor prognostic factors for LT.At LT, 43.3% (n=13) had complex/ adverse cytogenetics. 30% (n=9) had clonal progression compared to karyotype analysis at diagnosis (seven did not have prior cytogenetics results). Median survival after LT was 2.5months. A diagnosis of LT after 2009 (when azacytidine became available at our institution) was not shown to affect survival after LT (p=0.73). Outcomes were not different whether LT occurred below or above the age of 60years (p=0.73). One patient (3.3%) was lost to follow up. 63.3% (n=19) received supportive treatment including palliative chemotherapy. 16.7% (n=5) received azacytidine-based chemotherapy, while 13.3% (n=4) received an AML-type regimen/ allogeneic stem cell transplant and one (3.3%) received experimental treatment. Survival for the different treatment modalities was not statistically significant (p=0.19): palliative chemotherapy/ supportive care 1.5months, azacytidine-based 3.1months, intensive chemotherapy/ allogeneic stem cell transplant 8.1months.
Conclusion
As in published literature, outcomes for Asian MPN patients in blast phase are dismal. In this study, a presenting Hb <10g/dL and presenting WBC ≥ 13x109/L were predictors of LT in MPN. Existing treatment modalities are ineffective in attaining long term remission and have not improved survival over time.
Session topic: E-poster
Keyword(s): Leukemia, Myeloproliferative disorder, Outcome
Abstract: PB2020
Type: Publication Only
Background
Progression to acute leukaemia is a rare event in the natural history of the MPNs, namely essential thrombocythaemia (ET), polycythaemia vera (PV) and myelofibrosis. Prognosis in MPN patients with leukaemia transformation (LT) is almost universally fatal and no effective treatment is available.
Aims
As data on Asian patients with MPN in LT is lacking, we evaluated the risk factors and outcomes of Asian MPN patients with LT in this retrospective single centre analysis.
Methods
We reviewed the case records of 824 Asian patients with MPNs from our institution’s IRB approved MPN registry. Patients with LT were compared with other patients in the registry who did not have LT on follow up.
Results
Among 824 MPN patients, 30 were identified to have LT from 2002 to 2015. The median follow up was 6.2years. Median age of LT was 72years and occurred at a median of 33.2years after diagnosis. 3.9% (n=22) of ET, 0.5% (n=1) of PV and 11.5% (n=7) of myelofibrosis patients progressed to acute leukaemia (p <0.05). Time to LT was longest for ET (11years), followed by myelofibrosis (3.9years) and PV (1.5years) (p=0.018). There was no impact of JAK2, Calreticulin and MPL mutations on LT (p=0.55). Patients with LT had lower Hb at diagnosis (12.7g/dL vs. 14.8g/dL, p=0.001). A trend to higher presenting leukocyte count (15.5x 109/L vs. 12.6 x 109/L, p=0.053) and older age of diagnosis of MPN (59.5years vs. 56years, p=0.061) was seen in patients with LT (p=0.053).On log-rank test, MPN subtype (p<0.05), presenting Hb <10g/dL (p<0.05), presenting WBC ≥ 13x109/L (p=0.009), presenting platelet count ≥ 1000 x109/L (p=0.045) and age at diagnosis of MPN ≥ 60years (p=0.001) were factors for shorter time to LT. Use of hydroxyurea (p=0.17) and exposure to more than one cytoreductive agent (p=0.45) had no impact on LT. Using all co-variates with a p value ≤0.10, we developed a model using Cox proportional hazard model to determine the relative effect of these variables on LT. The model included the above variables and presence of splenomegaly at diagnosis (p=0.071). A presenting leukocyte count ≥ 13x109/L (HR 5.73, 95% Confidence Interval [CI] 1.84-17.88, p=0.03) and presenting Hb <10g/dL (HR 8.05, 95%CI 1.66-38.95, p=0.01) were independent poor prognostic factors for LT.At LT, 43.3% (n=13) had complex/ adverse cytogenetics. 30% (n=9) had clonal progression compared to karyotype analysis at diagnosis (seven did not have prior cytogenetics results). Median survival after LT was 2.5months. A diagnosis of LT after 2009 (when azacytidine became available at our institution) was not shown to affect survival after LT (p=0.73). Outcomes were not different whether LT occurred below or above the age of 60years (p=0.73). One patient (3.3%) was lost to follow up. 63.3% (n=19) received supportive treatment including palliative chemotherapy. 16.7% (n=5) received azacytidine-based chemotherapy, while 13.3% (n=4) received an AML-type regimen/ allogeneic stem cell transplant and one (3.3%) received experimental treatment. Survival for the different treatment modalities was not statistically significant (p=0.19): palliative chemotherapy/ supportive care 1.5months, azacytidine-based 3.1months, intensive chemotherapy/ allogeneic stem cell transplant 8.1months.
Conclusion
As in published literature, outcomes for Asian MPN patients in blast phase are dismal. In this study, a presenting Hb <10g/dL and presenting WBC ≥ 13x109/L were predictors of LT in MPN. Existing treatment modalities are ineffective in attaining long term remission and have not improved survival over time.
Session topic: E-poster
Keyword(s): Leukemia, Myeloproliferative disorder, Outcome
Type: Publication Only
Background
Progression to acute leukaemia is a rare event in the natural history of the MPNs, namely essential thrombocythaemia (ET), polycythaemia vera (PV) and myelofibrosis. Prognosis in MPN patients with leukaemia transformation (LT) is almost universally fatal and no effective treatment is available.
Aims
As data on Asian patients with MPN in LT is lacking, we evaluated the risk factors and outcomes of Asian MPN patients with LT in this retrospective single centre analysis.
Methods
We reviewed the case records of 824 Asian patients with MPNs from our institution’s IRB approved MPN registry. Patients with LT were compared with other patients in the registry who did not have LT on follow up.
Results
Among 824 MPN patients, 30 were identified to have LT from 2002 to 2015. The median follow up was 6.2years. Median age of LT was 72years and occurred at a median of 33.2years after diagnosis. 3.9% (n=22) of ET, 0.5% (n=1) of PV and 11.5% (n=7) of myelofibrosis patients progressed to acute leukaemia (p <0.05). Time to LT was longest for ET (11years), followed by myelofibrosis (3.9years) and PV (1.5years) (p=0.018). There was no impact of JAK2, Calreticulin and MPL mutations on LT (p=0.55). Patients with LT had lower Hb at diagnosis (12.7g/dL vs. 14.8g/dL, p=0.001). A trend to higher presenting leukocyte count (15.5x 109/L vs. 12.6 x 109/L, p=0.053) and older age of diagnosis of MPN (59.5years vs. 56years, p=0.061) was seen in patients with LT (p=0.053).On log-rank test, MPN subtype (p<0.05), presenting Hb <10g/dL (p<0.05), presenting WBC ≥ 13x109/L (p=0.009), presenting platelet count ≥ 1000 x109/L (p=0.045) and age at diagnosis of MPN ≥ 60years (p=0.001) were factors for shorter time to LT. Use of hydroxyurea (p=0.17) and exposure to more than one cytoreductive agent (p=0.45) had no impact on LT. Using all co-variates with a p value ≤0.10, we developed a model using Cox proportional hazard model to determine the relative effect of these variables on LT. The model included the above variables and presence of splenomegaly at diagnosis (p=0.071). A presenting leukocyte count ≥ 13x109/L (HR 5.73, 95% Confidence Interval [CI] 1.84-17.88, p=0.03) and presenting Hb <10g/dL (HR 8.05, 95%CI 1.66-38.95, p=0.01) were independent poor prognostic factors for LT.At LT, 43.3% (n=13) had complex/ adverse cytogenetics. 30% (n=9) had clonal progression compared to karyotype analysis at diagnosis (seven did not have prior cytogenetics results). Median survival after LT was 2.5months. A diagnosis of LT after 2009 (when azacytidine became available at our institution) was not shown to affect survival after LT (p=0.73). Outcomes were not different whether LT occurred below or above the age of 60years (p=0.73). One patient (3.3%) was lost to follow up. 63.3% (n=19) received supportive treatment including palliative chemotherapy. 16.7% (n=5) received azacytidine-based chemotherapy, while 13.3% (n=4) received an AML-type regimen/ allogeneic stem cell transplant and one (3.3%) received experimental treatment. Survival for the different treatment modalities was not statistically significant (p=0.19): palliative chemotherapy/ supportive care 1.5months, azacytidine-based 3.1months, intensive chemotherapy/ allogeneic stem cell transplant 8.1months.
Conclusion
As in published literature, outcomes for Asian MPN patients in blast phase are dismal. In this study, a presenting Hb <10g/dL and presenting WBC ≥ 13x109/L were predictors of LT in MPN. Existing treatment modalities are ineffective in attaining long term remission and have not improved survival over time.
Session topic: E-poster
Keyword(s): Leukemia, Myeloproliferative disorder, Outcome
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