Turkish Journal of Hematology最新文献

{"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}

{"title":"Rapid Relapse of Idiopathic Multicentric Castleman Disease After Siltuximab Discontinuation in a Case with Complete Remission for More Than 10 Years","authors":"Yu-Han Gao, Jian Li, Lu Zhang","doi":"10.4274/tjh.galenos.2024.2024.0339","DOIUrl":"10.4274/tjh.galenos.2024.2024.0339","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"283-285"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628763/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142584398","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":"Treatment Patterns and Clinical Outcomes in Patients with Hodgkin Lymphoma from Saudi Arabia, Türkiye, and South Africa: Subgroup Analysis from the International Multicenter Retrospective B-HOLISTIC Study","authors":"David Brittain, Saad Akhtar, Sylvia Rodrigues, Moosa Patel, Dhaya Moodley, Jaimendra Prithipal Singh, Lydia M Dreosti, Zainab Mohamed, Mubarak Al-Mansour, Mohsen Alzahrani, M Shahzad Rauf, Irfan Maghfoor, Sevgi Kalayoğlu Beşışık, Can Boğa, Güray Saydam, Zhongwen Huang, Yacob Pinchevsky, Burhan Ferhanoğlu","doi":"10.4274/tjh.galenos.2024.2024.0181","DOIUrl":"10.4274/tjh.galenos.2024.2024.0181","url":null,"abstract":"<p><strong>Objective: </strong>B-HOLISTIC was a real-world retrospective study of treatment patterns and clinical outcomes in Hodgkin lymphoma (HL) in regions outside Europe and North America. This subgroup analysis reports findings from Saudi Arabia, Türkiye, and South Africa.</p><p><strong>Materials and methods: </strong>Patients aged ≥18 years and diagnosed with stage IIB-IV classical HL receiving frontline chemotherapy (frontline cHL) and/or with relapsed/refractory HL (RRHL) from January 2010 to December 2013 were assessed. The primary endpoint was progression-free survival (PFS) in patients with RRHL.</p><p><strong>Results: </strong>Overall, 694 patients (RRHL: n=178; frontline cHL: n=653) were enrolled. Among patients with RRHL, >80% received first salvage chemotherapy. The most common first salvage regimens were etoposide, methylprednisolone, cytarabine, and cisplatin in Saudi Arabia (78.3%) and dexamethasone, cytarabine, and cisplatin in Türkiye (36.1%) and South Africa (40%). Median PFS (95% confidence interval [CI]) in the RRHL group was 5.1 (3.0-15.9), 19.7 (7.5-not reached), and 5.2 (1.1-10.1) months in Saudi Arabia, Türkiye, and South Africa, respectively. The 5-year PFS and overall survival (95% CI) rates in patients with RRHL were 33.2% (21.6-45.2) and 78.2% (65.9-86.5) in Saudi Arabia, 42.5% (29.5-54.9) and 79.4% (67.2-87.5) in Türkiye, and 13.1% (4.2-27.0) and 53% (35.5-67.8) in South Africa, respectively.</p><p><strong>Conclusion: </strong>This study showed that the clinical outcomes in Türkiye and Saudi Arabia were generally comparable with those of Western countries during the study period, although Saudi Arabia had lower PFS rates. Conversely, the clinical outcomes in South Africa were suboptimal, emphasizing the need for novel therapies and improved progression to stem cell transplantation. These data may serve as a control group for future studies in these countries and inform clinical decision-making.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"211-224"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628755/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142508869","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 Rare Cause of Dyspnea in a Patient with Multiple Myeloma: Tracheobronchial Amyloidosis","authors":"Furkan Ufuk, İclal Ocak","doi":"10.4274/tjh.galenos.2024.2024.0188","DOIUrl":"10.4274/tjh.galenos.2024.2024.0188","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"273-274"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628756/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141560897","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 CD56 and CD117 Double-Positivity as a Predictor of Poor Prognosis in Multiple Myeloma Patients: A Retrospective Analysis","authors":"Hakan Keski, Selim Merdan, Itır Ebru Zemheri","doi":"10.4274/tjh.galenos.2024.2024.0149","DOIUrl":"10.4274/tjh.galenos.2024.2024.0149","url":null,"abstract":"<p><strong>Objective: </strong>Despite advancements in treatment, multiple myeloma (MM) remains a challenging hematologic malignancy. It is crucial to stratify risk and perform prognostic assessment with various markers, including the expression of cluster of differentiation 56 (CD56) and cluster of differentiation 117 (CD117). However, the relationship of these markers with MM-related survival remains unclear. In this context, the objective of this study was to investigate the prognostic implications of CD56 and CD117 expression and associated clinical features in MM patients.</p><p><strong>Materials and methods: </strong>The population of this retrospective single-center study consisted of adult MM patients whose CD56 and CD117 expression levels were analyzed. Patients were divided into four groups according to their immunophenotypes: CD56⁺CD117^-, CD56^-CD117⁺, CD56⁺CD117⁺, and CD56^-CD117^-. These groups were compared in terms of demographic and clinical characteristics, response to treatment, and survival outcomes.</p><p><strong>Results: </strong>Of the 168 MM patients included in the study, CD56 positivity, CD117 positivity, CD56 and CD117 double positivity, and CD56 and CD117 double negativity were observed in 57.1%, 38.1%, 21.4%, and 26.2%, respectively. Patients with double positivity had significantly higher cytogenetic risk and significantly lower overall response rate (ORR) compared to other patients (p<0.001 for both). ORR and overall survival (OS) were significantly lower in CD56-positive patients than in CD56-negative patients (p=0.017 and p=0.004, respectively). Mortality rates were significantly higher in CD56-positive and CD117-positive patients than in double-negative patients (p<0.001 and p=0.002, respectively). Double-negative patients had significantly lower ORR and OS and higher mortality than others (p=0.001, p=0.002, and p<0.001, respectively). High cytogenetic risk was found to be an independent predictor of shorter OS (p>0.001).</p><p><strong>Conclusion: </strong>This study’s findings revealed that MM patients with CD56 and CD117 double positivity had poorer prognosis, lower ORR, shorter OS, and higher mortality.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"236-245"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628754/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142393706","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":"Unmasking Congenital Methemoglobinemia: A Novel <i>CYB5R</i> Mutation Discovered in an Adult with Symptomatic Polycythemia","authors":"Amiya Ranjan Nayak, Pratyusha Gudapati, Swapnil Tripathi, Jasmita Dass, Pradeep Kumar, Mukul Aggarwal","doi":"10.4274/tjh.galenos.2024.2024.0232","DOIUrl":"10.4274/tjh.galenos.2024.2024.0232","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"281-282"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628760/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141907784","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}