Turkish Journal of Hematology最新文献

{"title":"Antifungal Prophylaxis in Hypomethylating Agents Venetoclax Combination in AML-Turkish Society of Hematology Subcommittee on Infections and Supportive Therapies in Hematology Survey Study.","authors":"Tugcan Alp Kırkızlar, Vildan Ozkocaman","doi":"10.4274/tjh.galenos.2024.2024.0436","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0436","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142886053","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":"Duplication of the Long Arm of Both Chromosome 3 Leads to <i>MECOM</i> Rearrangement in Acute Myeloid Leukemia.","authors":"Shaobin Yang, Shiyang Ma, Xiaoyan Yan, Jingya Yao, Yani Lin","doi":"10.4274/tjh.galenos.2024.2024.0395","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0395","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142855625","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":"Eosinophilia with <i>STAT5B</i>N642H Mutation: A Heterogeneous Entity with Overlapping Morphological Features and Poor Outcome","authors":"Venkat Shashidhar, Aishwarya Karthikeyan, Anand Balakrishnan, Sudhanshi Raina, Jasmina Ahluwalia, Reena Das, Pankaj Malhotra, Sreejesh Sreedharanunni","doi":"10.4274/tjh.galenos.2024.2024.0204","DOIUrl":"10.4274/tjh.galenos.2024.2024.0204","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"275-278"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628759/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142112417","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 Rapidly Progressing Tumoral-Stage Mycosis Fungoides","authors":"Muzaffer Keklik, Salih Levent Çınar, Rıza Şimşek, Leylagül Kaynar","doi":"10.4274/tjh.galenos.2024.2024.0298","DOIUrl":"10.4274/tjh.galenos.2024.2024.0298","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"291-292"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628767/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142649125","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":"Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis","authors":"Ünal Ataş, Ozan Salim, Utku Iltar, Orhan Kemal Yücel, Ayşe Hilal Küçükdiler Eroğlu, Vedat Aslan, Murat Yıldırım, Özen Dedeoğlu, Sema Seçilmiş, Turgay Ulaş, Burak Deveci, Sedanur Karaman Gulsaran, Ayfer Gedük, Fatih Yaman, İbrahim Ethem Pınar, Senem Maral, Ahmet Sarıcı, Serhat Çelik, Hande Oğul, Sıdıka Gülkan Özkan, Aliihsan Gemici, Ahmet Kurşat Güneş, Anil Tombak, İrfan Yavaşoğlu, Volkan Karakuş, Melda Cömert, Tayfur Toptaş, Mehmet Sinan Dal, Rabin Saba, Hakkı Onur Kırkızlar, Özgür Mehtap, Eren Gündüz, Fahir Özkalemkaş, Murat Albayrak, İlhami Berber, Muzaffer Keklik, Nil Güler, Hasan Atilla Özkan, Ömür Gökmen Sevindik, Zahit Bolaman, Erdal Kurtoğlu, Meltem Aylı, Tülin Fıratlı Tuğlular, Fevzi Altuntaş, Levent Ündar","doi":"10.4274/tjh.galenos.2024.2023.0450","DOIUrl":"10.4274/tjh.galenos.2024.2023.0450","url":null,"abstract":"<p><strong>Objective: </strong>In this study, we aimed to obtain real-life data on the use of antimyeloma agents, which significantly increase overall survival (OS) in multiple myeloma (MM) patients, in primary plasma cell leukemia (pPCL) patients with poor prognosis.</p><p><strong>Materials and methods: </strong>Data from 53 patients who were diagnosed with pPCL between 2011 and 2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of the pPCL diagnosis, 20% leukocytes or ≥2x10⁹/L plasma cells in the peripheral blood was used as a diagnostic criterion.</p><p><strong>Results: </strong>The median age of the patients was 58 years and 23 (43.4%) patients were over 65 years of age. For first-line treatment, PI or IMID alone was used by 31 (58.5%) patients, and PI and IMID were used simultaneously by 15 (28.3%) patients. Additionally, 21 (39.6%) patients received transplantation and 13 (24.5%) patients received maintenance treatment. The median progression-free survival was 4 (range: 1-42) months. When patients whose primary disease was refractory to first-line therapy were excluded, the duration of treatment was 6.5 months. The median OS was 15 months with a median follow-up duration of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher β2-microglobulin levels and International Staging System stage 3 and non-transplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02, and p=0.008, respectively). The concomitant use of PIs and IMIDs, the addition of chemotherapy to induction therapy, and the response to induction therapy or maintenance therapy did not affect OS.</p><p><strong>Conclusion: </strong>In this study, as in previous similar studies, we could not see an increased survival trend in pPCL, which is observed in MM. New studies are needed for pPCL, which is likely to increase with new diagnostic criteria, based on current agents and information from MM.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"225-235"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628765/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142584399","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":"Dilemmas in the Diagnosis and Management of CML in Pakistan","authors":"Sahrish Khan, Muhammad Farooq Sabar, Mariyam Akbar, Abdul Waris","doi":"10.4274/tjh.galenos.2024.2024.0280","DOIUrl":"10.4274/tjh.galenos.2024.2024.0280","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"286-287"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628766/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142393705","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":"Role of the Hemostasis and Thrombosis Unit in the Management of Patients with Acquired Hemophilia A","authors":"Antonella Mameli, Francesco Marongiu, Lara Fenu, Maria Filomena Ruberto, Paola Schirru, Simona Cornacchini, Doris Barcellona","doi":"10.4274/tjh.galenos.2024.2024.0230","DOIUrl":"10.4274/tjh.galenos.2024.2024.0230","url":null,"abstract":"<p><strong>Objective: </strong>Acquired hemophilia A (AHA) is a rare autoimmune disease characterized by the presence of autoantibodies against coagulation factor VIII, leading to spontaneous hemorrhage in patients without a prior family or personal history of bleeding. This study describes the demographics, diagnosis, underlying disorders, bleeding characteristics, treatment, and outcomes of 41 AHA patients together with specific case reports.</p><p><strong>Materials and methods: </strong>Diagnosis and treatment of these patients occurred between 2005 and 2023. The median age at diagnosis was 67.8 (range: 15-93) years. Among the 41 patients, 10 (24%) cases were idiopathic, 4 (10%) were postpartum, 18 (44%) involved autoimmune diseases, and 9 (22%) involved a diagnosis of cancer.</p><p><strong>Results: </strong>The diagnostic delay exceeded 30 days in 15 of the 41 cases (36.5%). A total of 38 of the 41 (93%) patients presented with spontaneous bleeding, with mucocutaneous bleeding being the most common presentation (23/41, 56%). Four patients experienced postpartum bleeding. Clinical remission was achieved by 100% of patients and no patients died.</p><p><strong>Conclusion: </strong>Hemostatic and immunosuppressive therapy is essential in AHA, and it should be started as soon as possible in patients with bleeding. However, a significant delay in diagnosis was observed in these cases. The absence of mortality is likely attributable to the management of the disease within a specialized hemostasis and thrombosis unit, which offers a clinical ward, a specialized laboratory, and a dedicated ambulatory service. The Italian Society for the Study of Haemostasis and Thrombosis is working to secure recognition of this essential role in every hospital.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"264-270"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142508781","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":"Daratumumab and Eltrombopag for Pure Red Cell Aplasia Following ABO-Incompatible Allogeneic Hematopoietic Stem Cell Transplant for Acute Lymphoblastic Leukemia","authors":"Mehmet Sezgin Pepeler, Simten Dağdaş, Emel İşleyen, Gaye Katayıfçı, Aydan Kılıçarslan, Esra Özhamam, Muhammed Talha Adakul, Fahir Öztürk, Funda Ceran, Gülsüm Özet","doi":"10.4274/tjh.galenos.2024.2024.0231","DOIUrl":"10.4274/tjh.galenos.2024.2024.0231","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"288-290"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142649130","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":"Evaluation of Safety and Efficacy of Emicizumab Prophylaxis in Egyptian Pediatric Patients with Hemophilia A","authors":"Tamer Hassan, Marwa Zakaria, Manar Fathy, Ahmed Farag, Eman Abdelhady, Dalia Gameil, Mustafa Abu Hashem","doi":"10.4274/tjh.galenos.2024.2024.0220","DOIUrl":"10.4274/tjh.galenos.2024.2024.0220","url":null,"abstract":"<p><strong>Objective: </strong>Hemophilia A (HA) is a hereditary X-linked bleeding disorder secondary to deficiency of the clotting factor VIII (FVIII). Emicizumab is a monoclonal antibody that replaces the function of the activated FVIII and prevents bleeding in HA patients. Emicizumab is expected to ameliorate bleeding risk in those patients together with subsequent complications. However, there is a scarcity of data about its safety and efficacy in patients with HA. We aimed to evaluate the safety and efficacy of emicizumab prophylaxis in Egyptian pediatric patients with HA.</p><p><strong>Materials and methods: </strong>A prospective cohort study was carried out with 88 HA patients who received emicizumab prophylaxis. Breakthrough bleeding episodes and the annualized bleeding rate (ABR) were reported for all patients before and after emicizumab prophylaxis. Also, all adverse events during prophylaxis were documented to evaluate the safety of emicizumab.</p><p><strong>Results: </strong>Joint bleeds occurred in 94% of the patients. Among those patients, 58% had one target joint, 36.4% had more than one target joint, and 5.6% had no target joints. Furthermore, 17% of patients were positive for FVIII inhibitors. The median annualized joint bleeding rate was reduced remarkably after emicizumab prophylaxis (36 before versus 0 after emicizumab). The median ABR was 48 before emicizumab versus 0 after emicizumab. Eight patients experienced mild breakthrough bleeding episodes. The most common adverse events were local reactions at injection sites, headache, arthralgia, fever, and diarrhea.</p><p><strong>Conclusion: </strong>Prophylaxis using emicizumab was associated with a significantly lower bleeding rate in HA patients with and without inhibitors. The majority of patients had zero bleeds with emicizumab prophylaxis.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"256-263"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628768/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142018780","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":"Peripheral Hemophagocytosis and Leukemic Blasts from Urine in De Novo 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":"10.4274/tjh.galenos.2024.2024.0126","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"271-272"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628758/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}