Contributions
Abstract: PB2285
Type: Publication Only
Background
Recent advances in molecular diagnostics found that the majority of classic myeloproliferative neoplasms (MPNs) harbor characteristic driver mutations. The JAK2 protein transmits signals of various cytokine receptors, while CALR and MPL are involved only in the thrombopoietin receptor signaling. This may contribute to different cytokines-related symptoms that impact quality of life. The MPN Symptom Assessment Form total symptom score (MPN-SAF TSS) or MPN-10 score is a useful tool to assess symptom burden. However, the score has not been applied to clinical practice in Thailand.
Aims
To determine the MPN-10 scores and correlate them with clinical characteristics, genetic mutations and outcomes of MPNs patients.
Methods
The baseline characteristics, MPN-10 score and outcomes of MPNs patients diagnosed according to the WHO criteria at King Chulalongkorn Memorial Hospital, Thailand between 2014 and 2017 were reviewed.
Results
There were 146 patients enrolled. Forty-seven patients were diagnosed as polycythemia vera (PV), 72 as essential thrombocythemia (ET), and 27 as primary myelofibrosis (PMF). One hundred and thirty-two patients (90%) had driver mutations. PV patients harbored JAK2V617F mutation in 46 (97.8%) and JAK2exon12 mutation in 1 (2.1%). ET patients had JAK2V617F mutation in 41 (56.9%), CALR mutation in 17 (23.6%), MPL mutation in 1 (1.3%) and triple negative mutation in 11 (15.2%), while PMF harbored JAK2V617F mutation in 20 (74.0%), CALR mutation in 4 (14.8%) and triple negative mutation in 3 (11.1%).
Interestingly, the mean MPN-10 score of Thai PV and ET patients were significantly lower than those of Western population 11.7 ± 11.4 vs. 21.8 ± 16.3, p=0.002, and 10.7 ± 9.7 vs. 18.7 ± 15.3, p=0.0014 for PV and ET respectively. The mean MPN-10 score of Thai patients vs. Western MF patients was 24.4±12.5 vs. 25.3±17.2, respectively, p=0.862. In ET, the MPN-10 scores were not different between cases with JAK2 vs. CALR mutations. In addition, a MPN-10 score is not associated with survival (p=0.643).
Conclusion
PMF had a higher symptom burden than PV and ET. MPN-10 scores in Thai PV and ET were significantly lower than those of Western patients. MPN-10 scores were not associated with driver mutations and could not predict survival in Thai MPNs patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Essential Thrombocytemia, Myelofibrosis, Myeloproliferative disorder, Polycythemia vera
Abstract: PB2285
Type: Publication Only
Background
Recent advances in molecular diagnostics found that the majority of classic myeloproliferative neoplasms (MPNs) harbor characteristic driver mutations. The JAK2 protein transmits signals of various cytokine receptors, while CALR and MPL are involved only in the thrombopoietin receptor signaling. This may contribute to different cytokines-related symptoms that impact quality of life. The MPN Symptom Assessment Form total symptom score (MPN-SAF TSS) or MPN-10 score is a useful tool to assess symptom burden. However, the score has not been applied to clinical practice in Thailand.
Aims
To determine the MPN-10 scores and correlate them with clinical characteristics, genetic mutations and outcomes of MPNs patients.
Methods
The baseline characteristics, MPN-10 score and outcomes of MPNs patients diagnosed according to the WHO criteria at King Chulalongkorn Memorial Hospital, Thailand between 2014 and 2017 were reviewed.
Results
There were 146 patients enrolled. Forty-seven patients were diagnosed as polycythemia vera (PV), 72 as essential thrombocythemia (ET), and 27 as primary myelofibrosis (PMF). One hundred and thirty-two patients (90%) had driver mutations. PV patients harbored JAK2V617F mutation in 46 (97.8%) and JAK2exon12 mutation in 1 (2.1%). ET patients had JAK2V617F mutation in 41 (56.9%), CALR mutation in 17 (23.6%), MPL mutation in 1 (1.3%) and triple negative mutation in 11 (15.2%), while PMF harbored JAK2V617F mutation in 20 (74.0%), CALR mutation in 4 (14.8%) and triple negative mutation in 3 (11.1%).
Interestingly, the mean MPN-10 score of Thai PV and ET patients were significantly lower than those of Western population 11.7 ± 11.4 vs. 21.8 ± 16.3, p=0.002, and 10.7 ± 9.7 vs. 18.7 ± 15.3, p=0.0014 for PV and ET respectively. The mean MPN-10 score of Thai patients vs. Western MF patients was 24.4±12.5 vs. 25.3±17.2, respectively, p=0.862. In ET, the MPN-10 scores were not different between cases with JAK2 vs. CALR mutations. In addition, a MPN-10 score is not associated with survival (p=0.643).
Conclusion
PMF had a higher symptom burden than PV and ET. MPN-10 scores in Thai PV and ET were significantly lower than those of Western patients. MPN-10 scores were not associated with driver mutations and could not predict survival in Thai MPNs patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Essential Thrombocytemia, Myelofibrosis, Myeloproliferative disorder, Polycythemia vera