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
{"title":"CALR和JAK2V617F突变对重要血小板增多症临床过程和疾病结局的影响：土耳其患者的多中心回顾性研究。","authors":"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","doi":"10.4274/tjh.galenos.2024.2023.0430","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>In this study, we investigated the effects of calreticulin (<i>CALR</i>) and <i>JAK2</i>V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET).</p><p><strong>Materials and methods: </strong>Seventeen centers from Türkiye participated in the study and <i>CALR</i>- and <i>JAK2</i>V617F-mutated ET patients were evaluated retrospectively.</p><p><strong>Results: </strong>A total of 302 patients were included, of whom 203 (67.2%) and 99 (32.8%) were <i>JAK2</i>V617F- and <i>CALR</i>-positive, respectively. <i>CALR</i>-mutated patients were significantly younger (51 years vs. 57.5 years, p=0.03), with higher median platelet counts (987x10<sup>9</sup>/L vs. 709x10<sup>9</sup>/L, p<0.001) and lower median hemoglobin levels (13.1 g/dL vs. 14.1 g/dL, p<0.001) compared to <i>JAK2</i>V617F-mutated patients. Thromboembolic events (TEEs) occurred in 54 patients (17.9%), 77.8% of which were arterial. Compared to <i>CALR</i> mutation, <i>JAK2</i>V617F 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 <i>JAK2</i>V617F- and <i>CALR</i>-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 <i>JAK2</i>V617F- and <i>CALR</i>-mutated patients. Thrombosis-free survival (TFS) was superior in <i>CALR</i>-mutated patients compared to <i>JAK2</i>V617F-positive patients (5-year TFS: 90% vs. 71%, respectively; p=0.001). Age at diagnosis was an independent factor affecting the incidence of TEEs.</p><p><strong>Conclusion: </strong>In our ET cohort, <i>CALR</i> mutations resulted in higher platelet counts and lower hemoglobin levels than <i>JAK2</i>V617F and were associated with younger age at diagnosis. <i>JAK2</i>V617F was strongly associated with thrombosis and worse TFS. Hydroxyurea was the most preferred cytoreductive agent for patients with high thrombosis risk.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"41 1","pages":"26-36"},"PeriodicalIF":1.5000,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10918406/pdf/","citationCount":"0","resultStr":"{\"title\":\"Impact of <i>CALR</i> and <i>JAK2</i>V617F Mutations on Clinical Course and Disease Outcomes in Essential Thrombocythemia: A Multicenter Retrospective Study in Turkish Patients.\",\"authors\":\"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\",\"doi\":\"10.4274/tjh.galenos.2024.2023.0430\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objective: </strong>In this study, we investigated the effects of calreticulin (<i>CALR</i>) and <i>JAK2</i>V617F mutational status on clinical course and disease outcomes in Turkish patients with essential thrombocythemia (ET).</p><p><strong>Materials and methods: </strong>Seventeen centers from Türkiye participated in the study and <i>CALR</i>- and <i>JAK2</i>V617F-mutated ET patients were evaluated retrospectively.</p><p><strong>Results: </strong>A total of 302 patients were included, of whom 203 (67.2%) and 99 (32.8%) were <i>JAK2</i>V617F- and <i>CALR</i>-positive, respectively. <i>CALR</i>-mutated patients were significantly younger (51 years vs. 57.5 years, p=0.03), with higher median platelet counts (987x10<sup>9</sup>/L vs. 709x10<sup>9</sup>/L, p<0.001) and lower median hemoglobin levels (13.1 g/dL vs. 14.1 g/dL, p<0.001) compared to <i>JAK2</i>V617F-mutated patients. Thromboembolic events (TEEs) occurred in 54 patients (17.9%), 77.8% of which were arterial. Compared to <i>CALR</i> mutation, <i>JAK2</i>V617F 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 <i>JAK2</i>V617F- and <i>CALR</i>-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 <i>JAK2</i>V617F- and <i>CALR</i>-mutated patients. Thrombosis-free survival (TFS) was superior in <i>CALR</i>-mutated patients compared to <i>JAK2</i>V617F-positive patients (5-year TFS: 90% vs. 71%, respectively; p=0.001). Age at diagnosis was an independent factor affecting the incidence of TEEs.</p><p><strong>Conclusion: </strong>In our ET cohort, <i>CALR</i> mutations resulted in higher platelet counts and lower hemoglobin levels than <i>JAK2</i>V617F and were associated with younger age at diagnosis. <i>JAK2</i>V617F was strongly associated with thrombosis and worse TFS. Hydroxyurea was the most preferred cytoreductive agent for patients with high thrombosis risk.</p>\",\"PeriodicalId\":23362,\"journal\":{\"name\":\"Turkish Journal of Hematology\",\"volume\":\"41 1\",\"pages\":\"26-36\"},\"PeriodicalIF\":1.5000,\"publicationDate\":\"2024-03-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10918406/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Turkish Journal of Hematology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.4274/tjh.galenos.2024.2023.0430\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Turkish Journal of Hematology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.4274/tjh.galenos.2024.2023.0430","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"HEMATOLOGY","Score":null,"Total":0}
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 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.
期刊介绍：
The Turkish Journal of Hematology is published quarterly (March, June, September, and December) by the Turkish Society of Hematology. It is an independent, non-profit peer-reviewed international English-language periodical encompassing subjects relevant to hematology.
The Editorial Board of The Turkish Journal of Hematology adheres to the principles of the World Association of Medical Editors (WAME), International Council of Medical Journal Editors (ICMJE), Committee on Publication Ethics (COPE), Consolidated Standards of Reporting Trials (CONSORT) and Strengthening the Reporting of Observational Studies in Epidemiology (STROBE).
The aim of The Turkish Journal of Hematology is to publish original hematological research of the highest scientific quality and clinical relevance. Additionally, educational material, reviews on basic developments, editorial short notes, images in hematology, and letters from hematology specialists and clinicians covering their experience and comments on hematology and related medical fields as well as social subjects are published. As of December 2015, The Turkish Journal of Hematology does not accept case reports. Important new findings or data about interesting hematological cases may be submitted as a brief report.
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