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Impact of CALR and JAK2V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients.
Narli Özdemir, Zehra; Ipek, Yildiz; Patir, Püsem; Ermis, Gözde; Çiftçiler, Rafiye; Özmen, Deniz; Baysal, Mehmet; Gürsoy, Vildan; Yildizhan, Esra; Güven, Serkan; Ercan, Tarik; Elibol, Tayfun; Mersin, Sinan; Genç, Eylem; Davulcu, Eren Arslan; Karakus, Volkan; Erkut, Nergiz; Günes, Gürsel; Diz Küçükkaya, Reyhan; Eskazan, Ahmet Emre.
Affiliation
  • Narli Özdemir Z; Izmir City Hospital, Clinic of Hematology, Izmir, Türkiye.
  • Ipek Y; Kartal Dr. Lüfti Kirdar City Hospital, Clinic of Hematology, Istanbul, Türkiye.
  • Patir P; Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Türkiye.
  • Ermis G; Karadeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, Türkiye.
  • Çiftçiler R; Selçuk University Faculty of Medicine, Department of Hematology, Konya, Türkiye.
  • Özmen D; Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul, Türkiye.
  • Baysal M; Ali Osman Sönmez Oncology Hospital, Clinic of Hematology, Bursa, Türkiye.
  • Gürsoy V; Bursa City Hospital, Clinic of Orthopedics and Traumatology, Bursa, Türkiye.
  • Yildizhan E; Kayseri City Hospital, Clinic of Hematology, Kayseri, Türkiye.
  • Güven S; Mehmet Akif Ersoy State Hospital, Clinic of Hematology, Çanakkale, Türkiye.
  • Ercan T; Haydarpasa Numune Training and Research Hospital, Clinic of Hematology, Istanbul, Türkiye.
  • Elibol T; Medeniyet University Göztepe Training and Research Hospital, Clinic of Hematology, Istanbul, Türkiye.
  • Mersin S; Mugla Sitki Koçman University Training and Research Hospital, Clinic of Hematology, Mugla, Türkiye.
  • Genç E; Tekirdag Dr. Ismail Fehmi Cumalioglu City Hospital, Clinic of Hematology, Tekirdag, Türkiye.
  • Davulcu EA; University of Health Sciences Türkiye, Istanbul Bakirköy Dr. Sadi Konuk Training and Research Hospital, Clinic of Hematology, Istanbul, Türkiye.
  • Karakus V; Antalya Training and Research Hospital, Clinic of Hematology, Antalya, Türkiye.
  • Erkut N; Karadeniz Technical University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Trabzon, Türkiye.
  • Günes G; University of Health Sciences Türkiye, Diskapi Yildirim Beyazit Training and Research Hospital, Clinic of Hematology, Ankara, Türkiye.
  • Diz Küçükkaya R; Istanbul University Faculty of Science, Department of Molecular Biology and Genetics, Istanbul, Türkiye.
  • Eskazan AE; Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul, Türkiye.
Turk J Haematol ; 41(1): 26-36, 2024 Mar 01.
Article in En | MEDLINE | ID: mdl-38433449
ABSTRACT

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.
Subject(s)
Key words

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Thrombosis / Primary Myelofibrosis / Thrombocythemia, Essential Limits: Humans Country/Region as subject: Asia Language: En Journal: Turk J Haematol Year: 2024 Type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Thrombosis / Primary Myelofibrosis / Thrombocythemia, Essential Limits: Humans Country/Region as subject: Asia Language: En Journal: Turk J Haematol Year: 2024 Type: Article