?LINICAL FEATURES AND MOLECULAR MARKERS IN ESSENTIAL THROMBOCYTHEMIA
(Abstract release date: 05/19/16)
EHA Library. Zherniakova A. 06/09/16; 134932; PB2032

Dr. Anastasiia Zherniakova
Contributions
Contributions
Abstract
Abstract: PB2032
Type: Publication Only
Background
Particular molecular marker detection (JAK2 V617F, MPL, CALR) or its absence (triple-negative (TN)) in essential thrombocythemia (ET) can be served as a basis of different biological neoplasm behavior.
Aims
The aim of this study was to investigate interactions (differences) between the presence of each molecular marker, clinical features and course of ET.
Methods
One hundred and fifty ET patients, who had been diagnosed at our institution according to WHO 2008 criteria. The following parameters were assessed: age, gender, complete blood count, clinical symptoms (weakness, headache, dizziness, arthralgia, constitutional symptoms, erythromelalgia, splenomegaly), thrombotic complications and bleeding. Overall survival (OS) in ET patients with different molecular markers were analyzed by Kaplan-Meyer method and compared between groups. Log-rank, ANOVA Kruskal-Wallis test and the Chi-square test with Yate’s contingency correction were used for statistical analysis.
Results
Median age was 56 years (range 19-82), 110 patients (73%) was female. Median follow-up was 28 months (range 1-168). The following mutations were detected: JAK2V617F (JAK2+) n=115 (76,7%), MPL+ n=1 (0,7%), CALR+ n=14 (9,3%) and triple-negative (TN) molecular status was registered in n=20 (13,3%) patients. Complete blood count mean values (standard deviations) at initial ET diagnosis were:JAK2+: Hb 14.2 (17.6) g/dL, WBC 9.7 (3.7) х 109/L, PLT 831 (273) х109/L.MPL+: Hb 12.3 g/dL, WBC 7.1 х 109/L, PLT 2079 х109/L.CALR+: Hb 13.8 (16.7) g/dL, WBC 9.3 (3.9) х 109/L, PLT 1086 (453) х109/L.TN: Hb 13.6 (15.6) g/dL, WBC 11.4 (4.7) х 109/L, PLT 777 (230) х109/L.The presence of CALR mutation was associated with significant higher platelets level (p=0.03).The symptoms frequencies according molecular markers groups were as followed:JAK2+: weakness – 34.8%, headache/dizziness – 20.9%, arthralgia – 19.1%, splenomegaly -16.5%, erythromelalgia – 8.7%, pruritus – 5.2%, constitutional symptoms – 4.4%.MPL+: weakness only (100%).CALR+: weakness – 21.4%, headache/dizziness – 7.1%, splenomegaly – 21.4%, erythromelalgia – 14.3%.TN: weakness – 25.0%, headache/dizziness – 25.0%, arthralgia – 25.0%, splenomegaly – 10.0%, erythromelalgia – 10.0%, pruritus – 5.0%.Thrombosis rates according molecular markers groups were: JAK2+ - 48.6%; CALR+ - 14.2% - and TN – 30.0%. Bleeding frequency in CALR+ patients was 21.4% and for JAK2+ patients 6.9%. Overall survival (OS) was significant higher in CALR+ group compared to others (JAK2+ and TN, р=0.019).
Conclusion
The presence of JAK2V617F mutation in ET patients is associated with higher thrombosis risk, despite the fact of CALR+ patients had higher platelets level. OS in CALR+ ET patients was significant higher compared to JAK2+ and TN.

Session topic: E-poster
Keyword(s): Essential Thrombocytemia
Type: Publication Only
Background
Particular molecular marker detection (JAK2 V617F, MPL, CALR) or its absence (triple-negative (TN)) in essential thrombocythemia (ET) can be served as a basis of different biological neoplasm behavior.
Aims
The aim of this study was to investigate interactions (differences) between the presence of each molecular marker, clinical features and course of ET.
Methods
One hundred and fifty ET patients, who had been diagnosed at our institution according to WHO 2008 criteria. The following parameters were assessed: age, gender, complete blood count, clinical symptoms (weakness, headache, dizziness, arthralgia, constitutional symptoms, erythromelalgia, splenomegaly), thrombotic complications and bleeding. Overall survival (OS) in ET patients with different molecular markers were analyzed by Kaplan-Meyer method and compared between groups. Log-rank, ANOVA Kruskal-Wallis test and the Chi-square test with Yate’s contingency correction were used for statistical analysis.
Results
Median age was 56 years (range 19-82), 110 patients (73%) was female. Median follow-up was 28 months (range 1-168). The following mutations were detected: JAK2V617F (JAK2+) n=115 (76,7%), MPL+ n=1 (0,7%), CALR+ n=14 (9,3%) and triple-negative (TN) molecular status was registered in n=20 (13,3%) patients. Complete blood count mean values (standard deviations) at initial ET diagnosis were:JAK2+: Hb 14.2 (17.6) g/dL, WBC 9.7 (3.7) х 109/L, PLT 831 (273) х109/L.MPL+: Hb 12.3 g/dL, WBC 7.1 х 109/L, PLT 2079 х109/L.CALR+: Hb 13.8 (16.7) g/dL, WBC 9.3 (3.9) х 109/L, PLT 1086 (453) х109/L.TN: Hb 13.6 (15.6) g/dL, WBC 11.4 (4.7) х 109/L, PLT 777 (230) х109/L.The presence of CALR mutation was associated with significant higher platelets level (p=0.03).The symptoms frequencies according molecular markers groups were as followed:JAK2+: weakness – 34.8%, headache/dizziness – 20.9%, arthralgia – 19.1%, splenomegaly -16.5%, erythromelalgia – 8.7%, pruritus – 5.2%, constitutional symptoms – 4.4%.MPL+: weakness only (100%).CALR+: weakness – 21.4%, headache/dizziness – 7.1%, splenomegaly – 21.4%, erythromelalgia – 14.3%.TN: weakness – 25.0%, headache/dizziness – 25.0%, arthralgia – 25.0%, splenomegaly – 10.0%, erythromelalgia – 10.0%, pruritus – 5.0%.Thrombosis rates according molecular markers groups were: JAK2+ - 48.6%; CALR+ - 14.2% - and TN – 30.0%. Bleeding frequency in CALR+ patients was 21.4% and for JAK2+ patients 6.9%. Overall survival (OS) was significant higher in CALR+ group compared to others (JAK2+ and TN, р=0.019).
Conclusion
The presence of JAK2V617F mutation in ET patients is associated with higher thrombosis risk, despite the fact of CALR+ patients had higher platelets level. OS in CALR+ ET patients was significant higher compared to JAK2+ and TN.

Session topic: E-poster
Keyword(s): Essential Thrombocytemia
Abstract: PB2032
Type: Publication Only
Background
Particular molecular marker detection (JAK2 V617F, MPL, CALR) or its absence (triple-negative (TN)) in essential thrombocythemia (ET) can be served as a basis of different biological neoplasm behavior.
Aims
The aim of this study was to investigate interactions (differences) between the presence of each molecular marker, clinical features and course of ET.
Methods
One hundred and fifty ET patients, who had been diagnosed at our institution according to WHO 2008 criteria. The following parameters were assessed: age, gender, complete blood count, clinical symptoms (weakness, headache, dizziness, arthralgia, constitutional symptoms, erythromelalgia, splenomegaly), thrombotic complications and bleeding. Overall survival (OS) in ET patients with different molecular markers were analyzed by Kaplan-Meyer method and compared between groups. Log-rank, ANOVA Kruskal-Wallis test and the Chi-square test with Yate’s contingency correction were used for statistical analysis.
Results
Median age was 56 years (range 19-82), 110 patients (73%) was female. Median follow-up was 28 months (range 1-168). The following mutations were detected: JAK2V617F (JAK2+) n=115 (76,7%), MPL+ n=1 (0,7%), CALR+ n=14 (9,3%) and triple-negative (TN) molecular status was registered in n=20 (13,3%) patients. Complete blood count mean values (standard deviations) at initial ET diagnosis were:JAK2+: Hb 14.2 (17.6) g/dL, WBC 9.7 (3.7) х 109/L, PLT 831 (273) х109/L.MPL+: Hb 12.3 g/dL, WBC 7.1 х 109/L, PLT 2079 х109/L.CALR+: Hb 13.8 (16.7) g/dL, WBC 9.3 (3.9) х 109/L, PLT 1086 (453) х109/L.TN: Hb 13.6 (15.6) g/dL, WBC 11.4 (4.7) х 109/L, PLT 777 (230) х109/L.The presence of CALR mutation was associated with significant higher platelets level (p=0.03).The symptoms frequencies according molecular markers groups were as followed:JAK2+: weakness – 34.8%, headache/dizziness – 20.9%, arthralgia – 19.1%, splenomegaly -16.5%, erythromelalgia – 8.7%, pruritus – 5.2%, constitutional symptoms – 4.4%.MPL+: weakness only (100%).CALR+: weakness – 21.4%, headache/dizziness – 7.1%, splenomegaly – 21.4%, erythromelalgia – 14.3%.TN: weakness – 25.0%, headache/dizziness – 25.0%, arthralgia – 25.0%, splenomegaly – 10.0%, erythromelalgia – 10.0%, pruritus – 5.0%.Thrombosis rates according molecular markers groups were: JAK2+ - 48.6%; CALR+ - 14.2% - and TN – 30.0%. Bleeding frequency in CALR+ patients was 21.4% and for JAK2+ patients 6.9%. Overall survival (OS) was significant higher in CALR+ group compared to others (JAK2+ and TN, р=0.019).
Conclusion
The presence of JAK2V617F mutation in ET patients is associated with higher thrombosis risk, despite the fact of CALR+ patients had higher platelets level. OS in CALR+ ET patients was significant higher compared to JAK2+ and TN.

Session topic: E-poster
Keyword(s): Essential Thrombocytemia
Type: Publication Only
Background
Particular molecular marker detection (JAK2 V617F, MPL, CALR) or its absence (triple-negative (TN)) in essential thrombocythemia (ET) can be served as a basis of different biological neoplasm behavior.
Aims
The aim of this study was to investigate interactions (differences) between the presence of each molecular marker, clinical features and course of ET.
Methods
One hundred and fifty ET patients, who had been diagnosed at our institution according to WHO 2008 criteria. The following parameters were assessed: age, gender, complete blood count, clinical symptoms (weakness, headache, dizziness, arthralgia, constitutional symptoms, erythromelalgia, splenomegaly), thrombotic complications and bleeding. Overall survival (OS) in ET patients with different molecular markers were analyzed by Kaplan-Meyer method and compared between groups. Log-rank, ANOVA Kruskal-Wallis test and the Chi-square test with Yate’s contingency correction were used for statistical analysis.
Results
Median age was 56 years (range 19-82), 110 patients (73%) was female. Median follow-up was 28 months (range 1-168). The following mutations were detected: JAK2V617F (JAK2+) n=115 (76,7%), MPL+ n=1 (0,7%), CALR+ n=14 (9,3%) and triple-negative (TN) molecular status was registered in n=20 (13,3%) patients. Complete blood count mean values (standard deviations) at initial ET diagnosis were:JAK2+: Hb 14.2 (17.6) g/dL, WBC 9.7 (3.7) х 109/L, PLT 831 (273) х109/L.MPL+: Hb 12.3 g/dL, WBC 7.1 х 109/L, PLT 2079 х109/L.CALR+: Hb 13.8 (16.7) g/dL, WBC 9.3 (3.9) х 109/L, PLT 1086 (453) х109/L.TN: Hb 13.6 (15.6) g/dL, WBC 11.4 (4.7) х 109/L, PLT 777 (230) х109/L.The presence of CALR mutation was associated with significant higher platelets level (p=0.03).The symptoms frequencies according molecular markers groups were as followed:JAK2+: weakness – 34.8%, headache/dizziness – 20.9%, arthralgia – 19.1%, splenomegaly -16.5%, erythromelalgia – 8.7%, pruritus – 5.2%, constitutional symptoms – 4.4%.MPL+: weakness only (100%).CALR+: weakness – 21.4%, headache/dizziness – 7.1%, splenomegaly – 21.4%, erythromelalgia – 14.3%.TN: weakness – 25.0%, headache/dizziness – 25.0%, arthralgia – 25.0%, splenomegaly – 10.0%, erythromelalgia – 10.0%, pruritus – 5.0%.Thrombosis rates according molecular markers groups were: JAK2+ - 48.6%; CALR+ - 14.2% - and TN – 30.0%. Bleeding frequency in CALR+ patients was 21.4% and for JAK2+ patients 6.9%. Overall survival (OS) was significant higher in CALR+ group compared to others (JAK2+ and TN, р=0.019).
Conclusion
The presence of JAK2V617F mutation in ET patients is associated with higher thrombosis risk, despite the fact of CALR+ patients had higher platelets level. OS in CALR+ ET patients was significant higher compared to JAK2+ and TN.

Session topic: E-poster
Keyword(s): Essential Thrombocytemia
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