{"title":"Images of Temporomandibular Joint Arthropathy in a Severe Hemophilia B Patient with Inhibitor and a Type III Von Willebrand Patient with Inhibitor.","authors":"Selda Yenel, Dilek Aynur Çankal, Zühre Akarslan, Merve Yazol, Zühre Kaya","doi":"10.4274/tjh.galenos.2024.2024.0342","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0342","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142354705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mei Yang, Yujing Tian, Fengling Hou, Lin Zhong, Jingyan Li, Xiaohong Li
{"title":"Peripheral Hemophagocytosis and Leukemic Blasts from Urine in <i>de novo</i> Pure Erythroid Leukemia.","authors":"Mei Yang, Yujing Tian, Fengling Hou, Lin Zhong, Jingyan Li, Xiaohong Li","doi":"10.4274/tjh.galenos.2024.2024.0126","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0126","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142296499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Deficiency of Adenosine Deaminase 2","authors":"Çağrı Coşkun, Şule Ünal","doi":"10.4274/tjh.galenos.2024.2024.0265","DOIUrl":"10.4274/tjh.galenos.2024.2024.0265","url":null,"abstract":"<p><p>Adenosine deaminase 2 (<i>ADA2</i>) deficiency is an autosomal recessively inherited autoinflammatory disorder caused by loss-of-function mutations in the <i>ADA2</i> gene. Although the pathogenesis involves the triggering of a proinflammatory cascade due to increased production of inflammatory cytokines such as tumor necrosis factor (TNF)-α and dysregulation of neutrophil extracellular trap formation resulting from an excess accumulation of extracellular adenosine, the pathogenetic mechanism still needs further clarification due to the broad clinical spectrum. In addition to the initially described vasculitis-related symptoms, hematological, immunological, and autoinflammatory symptoms are now well recognized. The diagnosis is made by demonstration of pathogenic variants of <i>ADA2</i> with biallelic loss of function and identification of low plasma <i>ADA2</i> catalytic activity. Currently, TNF-α inhibitors are the treatment of choice for controlling vasculitis manifestations and preventing strokes. However, in patients presenting with severe hematologic findings, TNF-α inhibitors are not the treatment of choice and hematopoietic stem cell transplantation has been shown to be successful in selected cases. Recombinant <i>ADA2</i> protein and gene therapy are promising treatment modalities for the future. In conclusion, <i>ADA2</i> deficiency has a broad phenotype and should be considered in the differential diagnosis of different clinical situations. In this review, we summarize the disease manifestations of <i>ADA2</i> deficiency and available treatment options.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141907783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Furkan Ufuk, İclal Ocak, Lydia Chelala, Luis Landeras
{"title":"A Rare Cause of Giant Intrathoracic Mass in a Woman with Sickle Cell Disease: Extramedullary Hematopoiesis","authors":"Furkan Ufuk, İclal Ocak, Lydia Chelala, Luis Landeras","doi":"10.4274/tjh.galenos.2024.2024.0127","DOIUrl":"10.4274/tjh.galenos.2024.2024.0127","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140868251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sıdıka Gülkan Özkan, Ali Kimiaei, Ali Hakan Kaya, Mehmet Sezgin Pepeler, Hasan Atilla Özkan, Mutlu Arat
{"title":"Turkish Hematologists’ Preferences for Related Donor Selection: Results of a Multicenter Survey","authors":"Sıdıka Gülkan Özkan, Ali Kimiaei, Ali Hakan Kaya, Mehmet Sezgin Pepeler, Hasan Atilla Özkan, Mutlu Arat","doi":"10.4274/tjh.galenos.2024.2024.0099","DOIUrl":"10.4274/tjh.galenos.2024.2024.0099","url":null,"abstract":"<p><p>Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a widely utilized treatment for various hematological diseases. While selection criteria for unrelated donors are well established, there is a lack of consistency and standardization in the selection of related donors. This study investigated the current approach of hematologists to the selection of related donors at Turkish HSCT centers. The study employed a cross-sectional survey design, distributing a self-administered questionnaire to 95 adult and pediatric transplantation centers in Türkiye to investigate their approaches to related donor selection for allo-HSCT. The questionnaire collected data on various topics including the center’s experience in performing allo-HSCT, patient groups treated, number of allo-HSCT procedures conducted between 2015 and 2021, preferences for related donors, considerations in related donor selection (such as sex and past pregnancies), guidelines utilized for related donor selection, upper age limit for related donors, and the use of specialized advanced analyses for elderly donors. The response rate to the survey was 38.9%. Variability was observed across centers in terms of sex consideration and the impact of past pregnancies on related female donor rejection. Different guidelines were employed for related donor selection, with the European Bone Marrow Transplantation guidelines being the most commonly used. Regarding the upper age limit for related donors, 8.1% of centers accepted an upper age limit of 55 years, 48.7% preferred an upper age limit of 65 years, and 43.2% selected related donors aged 65 and above. The lack of standardized guidelines for related donor selection in HSCT centers leads to variability in criteria and potential risks. Collaboration among centers is essential to establish consensus and develop standardized protocols.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Obinutuzumab for the Treatment of Cold Agglutinin Disease: A Case Report","authors":"Siyuan Li, Kaini Shen, Lu Zhang","doi":"10.4274/tjh.galenos.2024.2024.0132","DOIUrl":"10.4274/tjh.galenos.2024.2024.0132","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":null,"pages":null},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141318423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}