Contributions
Abstract: PB2297
Type: Publication Only
Background
The prognostication of myeloproliferative neoplasm (MPN) has been always challenging even in the advent of Janus kinase 2 (JAK2 V617F) molecular studies. The survival pattern of MPN in developing country like Malaysia is still awaiting discovery.
Aims
To investigate the mortality outcome in relation to demographic, clinical presentation and clinical blood parameters.
Methods
This was a retrospective national registry conducted from year 2009 to 2015 in Malaysia with description of mortality outcome in correlation to demographic, MPN subtypes, JAK2 V617F mutation, thrombosis, haemorrhagic events and blood parameters.
Results
A total of 865 patients were included for survival analysis. The mean survival duration was estimated to be 23.2 years (95% CI:20.739, 25.684). There were 154 deaths with 57.8% of males and 42.2% of females. The ethnic distribution among the decease were 50% of Malay, 40.9% of Chinese, 7.1% of Indian and 1.9% of others. JAK2 V617F was present in 61.1% while absent in 14.9% of mortality cases. The causes of death were mainly non-haematological related (55.2%) while haematological related was only 23.4%. The MPN sub-types like essential thrombocythemia (ET) had the best mortality outcome followed by polycythemia vera (PV), hypereosinophilia syndrome (HES), MPN-unclassifiable (MPN-U) and primary myelofibrosis (PMF) which was associated with odds of death of 1.2 times in comparison to others, p<0.001. The JAK2 V617F mutation had no influence in the overall survival (OS). The haemoglobin <12g/dL, haematocrit <35%, platelet <150 x109/dL and white blood count <5 x109/dL were significantly associated with the inferior OS (p<0.001). The arterial or venous thrombosis had no effect on the OS while those with bleeding events had a marginally significant better outcome (p=0.04). The grade 2 and above bone marrow fibrosis had a worse OS compared to the grade 1 and below bone marrow fibrosis. Patients presented with vasomotor symptoms were found to do better than those patients without (p=0.003).
Conclusion
The ET had the best OS while PMF had the worst OS. This is in conjunction with low blood counts, worsening bone marrow fibrosis and without vasomotor symptoms. The survival outcome of the MPN registry is instrumental for future policy development of effective healthcare in Malaysia.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Mortality
Abstract: PB2297
Type: Publication Only
Background
The prognostication of myeloproliferative neoplasm (MPN) has been always challenging even in the advent of Janus kinase 2 (JAK2 V617F) molecular studies. The survival pattern of MPN in developing country like Malaysia is still awaiting discovery.
Aims
To investigate the mortality outcome in relation to demographic, clinical presentation and clinical blood parameters.
Methods
This was a retrospective national registry conducted from year 2009 to 2015 in Malaysia with description of mortality outcome in correlation to demographic, MPN subtypes, JAK2 V617F mutation, thrombosis, haemorrhagic events and blood parameters.
Results
A total of 865 patients were included for survival analysis. The mean survival duration was estimated to be 23.2 years (95% CI:20.739, 25.684). There were 154 deaths with 57.8% of males and 42.2% of females. The ethnic distribution among the decease were 50% of Malay, 40.9% of Chinese, 7.1% of Indian and 1.9% of others. JAK2 V617F was present in 61.1% while absent in 14.9% of mortality cases. The causes of death were mainly non-haematological related (55.2%) while haematological related was only 23.4%. The MPN sub-types like essential thrombocythemia (ET) had the best mortality outcome followed by polycythemia vera (PV), hypereosinophilia syndrome (HES), MPN-unclassifiable (MPN-U) and primary myelofibrosis (PMF) which was associated with odds of death of 1.2 times in comparison to others, p<0.001. The JAK2 V617F mutation had no influence in the overall survival (OS). The haemoglobin <12g/dL, haematocrit <35%, platelet <150 x109/dL and white blood count <5 x109/dL were significantly associated with the inferior OS (p<0.001). The arterial or venous thrombosis had no effect on the OS while those with bleeding events had a marginally significant better outcome (p=0.04). The grade 2 and above bone marrow fibrosis had a worse OS compared to the grade 1 and below bone marrow fibrosis. Patients presented with vasomotor symptoms were found to do better than those patients without (p=0.003).
Conclusion
The ET had the best OS while PMF had the worst OS. This is in conjunction with low blood counts, worsening bone marrow fibrosis and without vasomotor symptoms. The survival outcome of the MPN registry is instrumental for future policy development of effective healthcare in Malaysia.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Mortality