{"title":"Primary Lymphoma of the Lacrimal Gland on PET/CT Imaging","authors":"Ahmet Eren Şen, Mustafa Erol","doi":"10.4274/tjh.galenos.2024.2024.0152","DOIUrl":"10.4274/tjh.galenos.2024.2024.0152","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"190-191"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589372/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141559786","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"<i>JAK2</i>V617F Mutation in Endothelial Cells of Patients with Atherosclerotic Carotid Disease","authors":"Reyhan Diz-Küçükkaya, Taner İyigün, Özgür Albayrak, Candan Eker, Tuba Günel","doi":"10.4274/tjh.galenos.2024.2024.0161","DOIUrl":"10.4274/tjh.galenos.2024.2024.0161","url":null,"abstract":"<p><strong>Objective: </strong>It has been shown that clonal mutations occur in hematopoietic stem cells with advancing age and increase the risk of death due to atherosclerotic vascular diseases, similarly to myeloproliferative neoplasms. Endothelial cells (ECs) and hematopoietic stem cells develop from common stem cells called hemangioblasts in the early embryonic period. However, the presence of hemangioblasts in the postnatal period is controversial. In this study, <i>JAK2</i> gene variants were examined in patients with atherosclerotic carotid disease and without any hematological malignancies.</p><p><strong>Materials and methods: </strong>Ten consecutive patients (8 men and 2 women) with symptomatic atherosclerotic carotid stenosis were included in this study. ECs (CD31<sup>+</sup>CD45<sup>-</sup>) were separated from tissue samples taken by carotid endarterectomy. <i>JAK2</i> variants were examined in ECs, peripheral blood mononuclear cells, and oral epithelial cells of the patients with next-generation sequencing.</p><p><strong>Results: </strong>The median age of the patients was 74 (range: 58-80) years and the median body mass index value was 24.44 (range: 18.42-30.85) kg/m<sup>2</sup>. Smoking history was present in 50%, hypertension in 80%, diabetes in 70%, and ischemic heart disease in 70% of the cases. The <i>JAK2</i>V617F mutation was detected in the peripheral blood mononuclear cells of 3 of the 10 patients, and 2 patients also had the <i>JAK2</i>V617F mutation in their ECs. The <i>JAK2</i>V617F mutation was not found in the oral epithelial cells of any of the patients.</p><p><strong>Conclusion: </strong>In this study, for the first time in the literature, we showed that the <i>JAK2</i>V617F mutation was found somatically in both peripheral blood cells and ECs in patients with atherosclerosis. This finding may support that ECs and hematopoietic cells originate from a common clone or that somatic mutations can be transmitted to ECs by other mechanisms. Examining the molecular and functional changes caused by the <i>JAK2</i>V617F mutation in ECs may help open a new avenue for treating atherosclerosis.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"167-174"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589362/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155576","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management of Primary Immune Thrombocytopenia: Turkish Modified Delphi-Based Consensus Statement for Special Considerations","authors":"Elif Gülsüm Ümit, Ahmet Muzaffer Demir, Muhlis Cem Ar, Mesut Ayer, Meltem Aylı, Volkan Karakuş, Emin Kaya, Fahir Özkalemkaş, Nilgün Sayınalp, Mehmet Sönmez, Fahri Şahin, Selami Koçak Toprak, Tayfur Toptaş, İrfan Yavaşoğlu, Ümran Çalış","doi":"10.4274/tjh.galenos.2024.2024.0101","DOIUrl":"10.4274/tjh.galenos.2024.2024.0101","url":null,"abstract":"<p><strong>Objective: </strong>Primary immune thrombocytopenia (ITP) is an acquired disorder of platelets with a complex and unclear mechanism of increased immune destruction or impaired production of platelets. While the management of ITP is evolving, there is still a need for guidance, particularly in certain circumstances such as pregnancy, emergencies, or patients requiring co-medications. We aimed to determine the tendencies of hematologists in Türkiye in the event of such special considerations.</p><p><strong>Materials and methods: </strong>Applying a modified Delphi method, the Turkish National ITP Working Group, founded under the auspices of the Turkish Society of Hematology, developed a questionnaire consisting of statements regarding pregnancy, emergencies, and circumstances requiring co-treatment with antiaggregants or anticoagulants. A total of 107 hematologists working in university or state hospitals voted for their agreement or disagreement with the statements for two sequential rounds.</p><p><strong>Results: </strong>The participating hematologists reached an agreement on starting treatment for pregnant patients with platelets of less than 30x10<sup>9</sup>/L and delivery either vaginally or by cesarean section being safe at platelet counts above 50x10<sup>9</sup>/L. For emergencies and the rescue management of ITP, the panel agreed against the use of high-dose corticosteroids alone, preferring combinations with transfusions or intravenous immunoglobulin. For patients who require interventions, platelet counts of >50x10<sup>9</sup>/L were regarded as safe for low-risk procedures as well as co-treatment with antiplatelets or anticoagulants.</p><p><strong>Conclusion: </strong>As the National ITP Study Group, we have observed the need to increase the practice guidance regarding patients with primary ITP requiring additional treatments including invasive interventions and co-treatments for coagulation. Decisions on the management of ITP during pregnancy should be individualized. There is a lack of consensus on the thresholds of platelet counts as well as co-morbidities and co-medications. This lack of consensus may be due to variations in practices.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"141-145"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589376/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Qian Wang, Ling-Ji Zeng, Man Wang, Jian-Yu Weng, Jin-Lan Pan
{"title":"Identification of a Novel <i>MAPK1::BCR</i> Fusion Gene/t(9;22) (q34;q11) in a Case of Acute Promyelocytic Leukemia","authors":"Qian Wang, Ling-Ji Zeng, Man Wang, Jian-Yu Weng, Jin-Lan Pan","doi":"10.4274/tjh.galenos.2024.2024.0105","DOIUrl":"10.4274/tjh.galenos.2024.2024.0105","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"194-199"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589371/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141560899","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Case of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with Coexistence of the <i>JAK2V617F</i> Clone","authors":"Li-Li Han, Xia Yang, Haiping Dai, Junfeng Zhu","doi":"10.4274/tjh.galenos.2024.2023.0467","DOIUrl":"10.4274/tjh.galenos.2024.2023.0467","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"123-125"},"PeriodicalIF":1.5,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589259/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140289055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management of Adult Primary Immune Thrombocytopenia: Delphi-Based Consensus Recommendations","authors":"Ahmet Muzaffer Demir, Elif Gülsüm Ümit, Muhlis Cem Ar, Mesut Ayer, Meltem Aylı, Volkan Karakuş, Emin Kaya, Fahir Özkalemkaş, Nilgün Sayınalp, Mehmet Sönmez, Fahri Şahin, Selami Koçak Toprak, Tayfur Toptaş, İrfan Yavaşoğlu, Ümran Çalış","doi":"10.4274/tjh.galenos.2024.2024.0055","DOIUrl":"10.4274/tjh.galenos.2024.2024.0055","url":null,"abstract":"<p><strong>Objective: </strong>Primary immune thrombocytopenia (pITP) is an acquired autoimmune disorder related to the increased destruction and/or impaired production of platelets. Its diagnosis and management are challenging and require expertise and the interpretation of international consensus reports and guidelines with national variations in availability. We aimed to assess the agreement of hematologists in Türkiye on certain aspects of both first-line and second-line management of patients with pITP.</p><p><strong>Materials and methods: </strong>Applying a modified Delphi method, the Turkish National ITP Working Group (14 steering committee members), founded under the auspices of the Turkish Society of Hematology, developed a 21-item questionnaire consisting of statements regarding the first-line and second-line treatment of pITP. A total of 107 adult hematologists working in either university or state hospitals voted for their agreement or disagreement with the statements in two consecutive rounds.</p><p><strong>Results: </strong>The participants reached consensus on the use of corticosteroids as first-line treatment and with limited duration. Methylprednisolone was the corticosteroid of choice rather than dexamethasone. Use of intravenous immunoglobulin was not preferred for patients without bleeding. It was also agreed that thrombopoietin receptor antagonists (TPO-RAs) or rituximab should be recommended as second-line treatment and that splenectomy could be considered 12-24 months after diagnosis in patients with chronic pITP.</p><p><strong>Conclusion: </strong>The optimization of the dose and duration of TPO-RAs in addition to corticosteroids is necessary to improve the management of patients with pITP.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"97-104"},"PeriodicalIF":1.5,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589261/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140307069","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Visceral Leishmaniasis Case from the Black Sea Region: Skin Lesions and <i>Leishmania donovani</i> Amastigotes in the Bone Marrow","authors":"Birgül Öneç, Cihadiye Elif Öztürk, Ayten Yazıcı","doi":"10.4274/tjh.galenos.2023.2023.0254","DOIUrl":"10.4274/tjh.galenos.2023.2023.0254","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"118-120"},"PeriodicalIF":1.5,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589255/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9764313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
İlhan Koyuncu, Betül Koyuncu, Mehmet Can Uğur, Emin Koyun, Oktay Şenöz, Mustafa Doğduş, Oktay Bilgir
{"title":"A New Scoring System for the Evaluation of Ibrutinib-Associated Arrhythmias in Chronic Lymphocytic Leukemia: The ACEF Score","authors":"İlhan Koyuncu, Betül Koyuncu, Mehmet Can Uğur, Emin Koyun, Oktay Şenöz, Mustafa Doğduş, Oktay Bilgir","doi":"10.4274/tjh.galenos.2024.2024.0045","DOIUrl":"10.4274/tjh.galenos.2024.2024.0045","url":null,"abstract":"<p><strong>Objective: </strong>Bruton tyrosine kinase inhibition in cardiac tissue causes inhibition of the PI3K-AKT signaling pathway, which is responsible for protecting cardiac tissue during stress. Therefore, there is an increase in the risk of arrhythmia. This study explores the prediction of that risk with the Age-Creatinine-Ejection Fraction (ACEF) score as a simple scoring system based on the components of age, creatinine, and ejection fraction.</p><p><strong>Materials and methods: </strong>Patients diagnosed with chronic lymphocytic leukemia (CLL) and receiving ibrutinib treatment for at least 1 year were evaluated with echocardiography and Holter electrocardiography and the results were compared with a control group of CLL patients who had not received treatment. ACEF score was calculated with the formula age/left ventricular ejection fraction+1 (if creatinine >2.0 mg/dL).</p><p><strong>Results: </strong>When the arrhythmia development of the patients was evaluated, no statistically significant difference was found between the control and ibrutinib groups in terms of types of arrhythmias other than paroxysmal atrial fibrillation (PAF). PAF was found to occur at rates of 8% versus 22% (p=0.042) among ibrutinib non-users versus users. For patients using ibrutinib, an ACEF score of >1.21 predicted the development of PAF with 77% sensitivity and 75% specificity (area under the curve: 0.830, 95% confidence interval: 0.698-0.962, p<0.001).</p><p><strong>Conclusion: </strong>The ACEF score can be used as a risk score that predicts the development of PAF in patients diagnosed with CLL who are scheduled to start ibrutinib.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"91-96"},"PeriodicalIF":1.5,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589260/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140892705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Andrés Felipe Melo Arias, Silvia Escribano Serrat, Marta Polo Zarzuela, Cristina García Sánchez, Miguel Gómez Álvarez, Eduardo Anguita, Celina Benavente Cuesta, Fernando Ataúlfo González Fernández
{"title":"Acute Promyelocytic Leukemia with Basophilic Differentiation: A Rare Variant","authors":"Andrés Felipe Melo Arias, Silvia Escribano Serrat, Marta Polo Zarzuela, Cristina García Sánchez, Miguel Gómez Álvarez, Eduardo Anguita, Celina Benavente Cuesta, Fernando Ataúlfo González Fernández","doi":"10.4274/tjh.galenos.2023.2023.0344","DOIUrl":"10.4274/tjh.galenos.2023.2023.0344","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"113-115"},"PeriodicalIF":1.5,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589258/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138809263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}