Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients
Objective: In this study, we investigated the effects of calreticulin (CALR) and JAK2V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET). Materials and Methods: Seventeen centers from Türkiye participated in the study and CALR- and...
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| Format: | Article |
| Language: | English |
| Published: |
Galenos Publishing House
2024-03-01
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| Series: | Turkish Journal of Hematology |
| Subjects: | |
| Online Access: | https://tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-45538&look4= |
| _version_ | 1827323016179089408 |
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| author | Zehra Narlı Özdemir Yıldız İpek Püsem Patır Gözde Ermiş Rafiye Çiftçiler Deniz Özmen Mehmet Baysal Vildan Gürsoy Esra Yıldızhan Serkan Güven Tarık Ercan Tayfun Elibol Sinan Mersin Eylem Genç Eren Arslan Davulcu Volkan Karakuş Nergiz Erkut Gürsel Güneş Reyhan Diz Küçükkaya Ahmet Emre Eşkazan |
| author_facet | Zehra Narlı Özdemir Yıldız İpek Püsem Patır Gözde Ermiş Rafiye Çiftçiler Deniz Özmen Mehmet Baysal Vildan Gürsoy Esra Yıldızhan Serkan Güven Tarık Ercan Tayfun Elibol Sinan Mersin Eylem Genç Eren Arslan Davulcu Volkan Karakuş Nergiz Erkut Gürsel Güneş Reyhan Diz Küçükkaya Ahmet Emre Eşkazan |
| author_sort | Zehra Narlı Özdemir |
| collection | DOAJ |
| description | Objective: In this study, we investigated the effects of calreticulin (CALR) and JAK2V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET).
Materials and Methods: Seventeen centers from Türkiye participated in the study and CALR- and JAK2V617F-mutated ET patients were evaluated retrospectively.
Results: A total of 302 patients were included, of whom 203 (67.2%) and 99 (32.8%) were JAK2V617F- and CALR-positive, respectively. CALR-mutated patients were significantly younger (51 years vs. 57.5 years, p=0.03), with higher median platelet counts (987x109/L vs. 709x109/L, p<0.001) and lower median hemoglobin levels (13.1 g/dL vs. 14.1 g/dL, p<0.001) compared to JAK2V617F-mutated patients. Thromboembolic events (TEEs) occurred in 54 patients (17.9%), 77.8% of which were arterial. Compared to CALR mutation, JAK2V617F was associated with a higher risk of thrombosis (8.1% vs. 22.7%, p=0.002). Rates of transformation to myelofibrosis (MF) and leukemia were 4% and 0.7%, respectively, and these rates were comparable between JAK2V617F- and CALR-mutated cases. The estimated overall survival (OS) and MF-free survival of the entire cohort were 265.1 months and 235.7 months, respectively. OS and MF-free survival durations were similar between JAK2V617F- and CALR-mutated patients. Thrombosis-free survival (TFS) was superior in CALR-mutated patients compared to JAK2V617F-positive patients (5-year TFS: 90% vs. 71%, respectively; p=0.001). Age at diagnosis was an independent factor affecting the incidence of TEEs.
Conclusion: In our ET cohort, CALR mutations resulted in higher platelet counts and lower hemoglobin levels than JAK2V617F and were associated with younger age at diagnosis. JAK2V617F was strongly associated with thrombosis and worse TFS. Hydroxyurea was the most preferred cytoreductive agent for patients with high thrombosis risk. |
| first_indexed | 2024-04-25T01:39:13Z |
| format | Article |
| id | doaj.art-ab5f757b27aa42b59fc159ec0b70c9e6 |
| institution | Directory Open Access Journal |
| issn | 1308-5263 |
| language | English |
| last_indexed | 2024-04-25T01:39:13Z |
| publishDate | 2024-03-01 |
| publisher | Galenos Publishing House |
| record_format | Article |
| series | Turkish Journal of Hematology |
| spelling | doaj.art-ab5f757b27aa42b59fc159ec0b70c9e62024-03-08T08:06:42ZengGalenos Publishing HouseTurkish Journal of Hematology1308-52632024-03-01411263610.4274/tjh.galenos.2024.2023.0430Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish PatientsZehra Narlı Özdemir0https://orcid.org/0000-0003-3237-320XYıldız İpek1https://orcid.org/0000-0003-2952-2286Püsem Patır2https://orcid.org/0000-0001-5201-4680Gözde Ermiş3https://orcid.org/0000-0002-6638-7020Rafiye Çiftçiler4https://orcid.org/0000-0001-5687-8531Deniz Özmen5https://orcid.org/0000-0001-7561-446XMehmet Baysal6https://orcid.org/0000-0001-7681-4623Vildan Gürsoy7https://orcid.org/0000-0002-3645-9345Esra Yıldızhan8https://orcid.org/0000-0002-1026-5761Serkan Güven9https://orcid.org/0000-0001-8933-1081Tarık Ercan10https://orcid.org/0000-0003-4037-4106Tayfun Elibol11https://orcid.org/0000-0001-8738-5246Sinan Mersin12https://orcid.org/0000-0001-7588-6000Eylem Genç13https://orcid.org/0000-0003-3259-0060Eren Arslan Davulcu14https://orcid.org/0000-0001-9262-2883Volkan Karakuş15https://orcid.org/0000-0001-9178-2850Nergiz Erkut16Gürsel Güneş17https://orcid.org/0000-0003-2330-7753Reyhan Diz Küçükkaya18https://orcid.org/0000-0001-5814-7118Ahmet Emre Eşkazan19https://orcid.org/0000-0001-9568-0894İzmir City Hospital, Clinic of Hematology, İzmir, TürkiyeKartal Dr. Lüfti Kırdar City Hospital, Clinic of Hematology, İstanbul, TürkiyeAntalya Training and Research Hospital, Clinic of Hematology, Antalya, TürkiyeKaradeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, TürkiyeSelçuk University Faculty of Medicine, Department of Hematology, Konya, Türkiyeİstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, TürkiyeAli Osman Sönmez Oncology Hospital, Clinic of Hematology, Bursa, TürkiyeBursa City Hospital, Clinic of Orthopedics and Traumatology, Bursa, TürkiyeKayseri City Hospital, Clinic of Hematology, Kayseri, TürkiyeMehmet Akif Ersoy State Hospital, Clinic of Hematology, Çanakkale, TürkiyeHaydarpaşa Numune Training and Research Hospital, Clinic of Hematology, İstanbul, TürkiyeMedeniyet University Göztepe Training and Research Hospital, Clinic of Hematology, İstanbul, TürkiyeMuğla Sıtkı Koçman University Training and Research Hospital, Clinic of Hematology, Muğla, TürkiyeTekirdağ Dr. İsmail Fehmi Cumalıoğlu City Hospital, Clinic of Hematology, Tekirdağ, TürkiyeUniversity of Health Sciences Türkiye, İstanbul Bakırköy Dr. Sadi Konuk Training and Research Hospital, Clinic of Hematology, İstanbul, TürkiyeAntalya Training and Research Hospital, Clinic of Hematology, Antalya, TürkiyeKaradeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, TürkiyeUniversity of Health Sciences Türkiye, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Clinic of Hematology, Ankara, Türkiyeİstanbul University Faculty of Science, Department of Molecular Biology and Genetics, İstanbul, Türkiyeİstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, İstanbul, TürkiyeObjective: In this study, we investigated the effects of calreticulin (CALR) and JAK2V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET). Materials and Methods: Seventeen centers from Türkiye participated in the study and CALR- and JAK2V617F-mutated ET patients were evaluated retrospectively. Results: A total of 302 patients were included, of whom 203 (67.2%) and 99 (32.8%) were JAK2V617F- and CALR-positive, respectively. CALR-mutated patients were significantly younger (51 years vs. 57.5 years, p=0.03), with higher median platelet counts (987x109/L vs. 709x109/L, p<0.001) and lower median hemoglobin levels (13.1 g/dL vs. 14.1 g/dL, p<0.001) compared to JAK2V617F-mutated patients. Thromboembolic events (TEEs) occurred in 54 patients (17.9%), 77.8% of which were arterial. Compared to CALR mutation, JAK2V617F was associated with a higher risk of thrombosis (8.1% vs. 22.7%, p=0.002). Rates of transformation to myelofibrosis (MF) and leukemia were 4% and 0.7%, respectively, and these rates were comparable between JAK2V617F- and CALR-mutated cases. The estimated overall survival (OS) and MF-free survival of the entire cohort were 265.1 months and 235.7 months, respectively. OS and MF-free survival durations were similar between JAK2V617F- and CALR-mutated patients. Thrombosis-free survival (TFS) was superior in CALR-mutated patients compared to JAK2V617F-positive patients (5-year TFS: 90% vs. 71%, respectively; p=0.001). Age at diagnosis was an independent factor affecting the incidence of TEEs. Conclusion: In our ET cohort, CALR mutations resulted in higher platelet counts and lower hemoglobin levels than JAK2V617F and were associated with younger age at diagnosis. JAK2V617F was strongly associated with thrombosis and worse TFS. Hydroxyurea was the most preferred cytoreductive agent for patients with high thrombosis risk.https://tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-45538&look4=calr mutationessential thrombocythemiajak2v617f mutationmyeloproliferative neoplasm |
| spellingShingle | Zehra Narlı Özdemir Yıldız İpek Püsem Patır Gözde Ermiş Rafiye Çiftçiler Deniz Özmen Mehmet Baysal Vildan Gürsoy Esra Yıldızhan Serkan Güven Tarık Ercan Tayfun Elibol Sinan Mersin Eylem Genç Eren Arslan Davulcu Volkan Karakuş Nergiz Erkut Gürsel Güneş Reyhan Diz Küçükkaya Ahmet Emre Eşkazan Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients Turkish Journal of Hematology calr mutation essential thrombocythemia jak2v617f mutation myeloproliferative neoplasm |
| title | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients |
| title_full | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients |
| title_fullStr | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients |
| title_full_unstemmed | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients |
| title_short | Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients |
| title_sort | impact of calr and jak2v617f mutations on clinical course and disease outcomes in essential thrombocythemia a multicenter retrospective study in turkish patients |
| topic | calr mutation essential thrombocythemia jak2v617f mutation myeloproliferative neoplasm |
| url | https://tjh.com.tr/jvi.aspx?pdir=tjh&plng=eng&un=TJH-45538&look4= |
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