Turkish Journal of Hematology最新文献

{"title":"The Curious Tale of a Missing Bone Segment","authors":"Amiya Ranjan Nayak, Neelabh Nayan, Priyanka Naranje, Pradeep Kumar, Jasmita Dass, Mukul Aggarwal","doi":"10.4274/tjh.galenos.2024.2024.0039","DOIUrl":"10.4274/tjh.galenos.2024.2024.0039","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"205-206"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589374/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140094660","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":"Myeloproliferative Neoplasm Symptom Assessment Total Symptom Score (MPN-SAF TSS) in Chronic Myeloproliferative Neoplasms with Relation to Genetic Burden and Thrombosis","authors":"Elif Gülsüm Ümit, Mehmet Baysal, Hakkı Onur Kırkızlar, Ahmet Muzaffer Demir","doi":"10.4274/tjh.galenos.2024.2024.0011","DOIUrl":"10.4274/tjh.galenos.2024.2024.0011","url":null,"abstract":"<p><p>The Myeloproliferative Neoplasm Symptom Assessment Total Symptom Score (MPN-SAF TSS) is a surrogate marker for symptom evaluation in chronic myeloproliferative neoplasms (MPNs). However, insufficient data are available regarding the relationship among the MPN-SAF TSS, <i>JAK2</i> mutation allele burden, and thrombosis. In this retrospective analysis, we aimed to determine the genetic burdens, clinical features, and relationships with MPN-SAF TSS in MPN patients. One hundred thirty <i>JAK2</i>V617F-positive patients with MPNs were included in our study. We calculated the MPN-SAF TSS for all patients and compared it with their clinical characteristics. Patients with higher <i>JAK2</i>V617F mutation allele burden had higher MPN-SAF TSS values (p=0.008). Patients with thrombosis had higher MPN-SAF TSS than patients without thrombosis (p=0.003). The mean MPN-SAF TSS was higher in patients with primary myelofibrosis compared to those with polycythemia vera and essential thrombocythemia. Thrombosis was associated with increased symptom severity in several domains, including fatigue, abdominal discomfort, inactivity, night sweats, pruritus, weight loss, and early satiety. Additionally, an increase in <i>JAK2</i> allele burden was observed with higher symptom scores. The MPN-SAF TSS proved to be a reliable tool for assessing symptom burden in Turkish MPN patients. Furthermore, the significant association between thrombosis occurrence and symptom severity suggests that thrombotic events may contribute to symptom development. Notably, increasing <i>JAK2</i> allele burden was correlated with more severe symptoms, highlighting its potential role in predicting disease burden. This study emphasizes the importance of symptom assessment in MPN patients and supports the incorporation of the MPN-SAF TSS in routine clinical practice to enhance patient care and management.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"175-181"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589363/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155640","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":"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⁺CD45^-) 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/m². 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":"A Sole p.G391E Mutation in <i>PML::RARA</i> Identified in Relapsed Acute Promyelocytic Leukemia","authors":"Zhan Su, Tingxuan Wang, Wei Yu, Xiaolin Ma, Huishou Fan, Xiangcong Yin, Wei Wang","doi":"10.4274/tjh.galenos.2024.2024.0157","DOIUrl":"10.4274/tjh.galenos.2024.2024.0157","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"200-201"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589361/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141560898","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 30x10⁹/L and delivery either vaginally or by cesarean section being safe at platelet counts above 50x10⁹/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 >50x10⁹/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}

{"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}