Turkish Journal of Hematology最新文献

{"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":"https://doi.org/10.4274/tjh.galenos.2024.2024.0298","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Daratumumab and Eltrombopag for Pure Red Cell Aplasia Post ABO Incompatible Allogeneic Hematopoietic Stem Cell Transplant for Acute Lymphoblastic Leukemia.","authors":"Mehmet Sezgin Pepeler, Simten Dagdas, 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":"https://doi.org/10.4274/tjh.galenos.2024.2024.0231","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Late Effects After Hematopoietic Stem Cell Transplantation Among Childhood Transplant Survivors with Fanconi Anemia.","authors":"Cansu Ozkocer, Fatma Visal Okur, Huseyin Demirbilek, Burak Altintas, Nevin Cetin, Bulent Baris Kuskonmaz, Bora Gulhan, Hayrettin Hakan Aykan, Hulya Demir, Deniz Dogru Ersoz, Ugur Canpolat, Hasan Serkan Dogan, Elmas Nazli Gonc, Hatice Yasemin Balaban, Gurkan Bozdag, Sule Unal, Sevkiye Selin Aytac Eyupoglu, Rezan Topaloglu, Zeynep Alev Ozon, Fatma Gumruk, Duygu Uckan Cetinkaya","doi":"10.4274/tjh.galenos.2024.2024.0189","DOIUrl":"10.4274/tjh.galenos.2024.2024.0189","url":null,"abstract":"<p><strong>Background: </strong>Fanconi anemia is the most common inherited bone marrow failure syndrome. HSCT remains the only curative treatment for hematological manifestations of FA. Despite restoration of long-term hematopoiesis, patients continue to remain at risk of late effects.</p><p><strong>Objectives: </strong>In our study, we aimed to reveal the problems that occur in the long-term follow-up of FA patients, and point out an ongoing need for the improvement of long-term follow-up guidelines for childhood transplant survivors with FA.</p><p><strong>Study design: </strong>In this single centered, cross-sectional study, we analyzed the long-term outcome of 36 patients with FA according to current recommendations with a median age of 18.1 years (range: 6.1-36 years, male/female, 24/12) who underwent a HSCT at Pediatric Bone Marrow Transplantation (BMT) Unit between 1995 and 2019 and survived at least one year post-transplant.</p><p><strong>Results: </strong>The median long-term follow-up time was 8 years (range, 1-25 years). Gonadal dysfunction was detected in about 35% of our patients. 31% of the patients had hypergonadotropic hypogonadism, 4 % had hypogonadotropic hypogonadism. When the patients were evaluated for growth impairment, 7 of 12 patients who reached their final adult height and 12 of 21 patients who didn't complete their growth, had height standard deviation score below -2 SD. Three patients (9%) developed subclinical hypothyroidism, two (6%) had overt hypothyroidism and one (3%) had central hypothyroidism. Although, none of our patients fully met the criteria for metabolic syndrome, 23% of the patients had insulin resistance and 39% had dyslipidemia. Evaluation of organ dysfunctions revealed that almost 50% of the patients had obstructive and 21 % had restrictive changes in their pulmonary function tests. Hepatosteatosis was detected in 15% of the patients and mild valve dysfunction was detected in 50 % of evaluable patients. Three patients developed secondary malignancies. Squamous cell cancer developed in 2 patients and basal cell cancer in one patient.</p><p><strong>Conclusion: </strong>A risk-defined multidisciplinary approach for long-term follow up of children with FA undergoing HSCT is essential for early detection and management of late effects.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142606466","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":"Survival Outcomes of Patients with Primary Plasma Cell Leukemia in the Era of Proteasome Inhibitors and Immunomodulatory Agents: A Real-Life Multicenter Analysis.","authors":"Unal Atas, Ozan Salim, Utku Iltar, Orhan Kemal Yucel, Ayse Hilal Kucukdiler Eroglu, Vedat Aslan, Murat Yildirim, Ozen Dedeoglu, Sema Secilmis, Turgay Ulas, Burak Deveci, Sedanur Karaman Gulsaran, Ayfer Geduk, Fatih Yaman, Ibrahim Ethem Pinar, Senem Maral, Ahmet Sarici, Serhat Celik, Hande Ogul, Sidika Gulkan Ozkan, Aliihsan Gemici, Ahmet Kursat Gunes, Anil Tombak, Irfan Yavasoglu, Volkan Karakus, Melda Comert, Tayfur Toptas, Mehmet Sinan Dal, Rabin Saba, Hakki Onur Kirgizlar, Ozgur Mehtap, Eren Gunduz, Fahir Ozkalemkas, Murat Albayrak, Ilhami Berber, Muzaffer Keklik, Nil Guler, Hasan Atilla Ozkan, Omur Gokmen Sevindik, Zahit Bolaman, Erdal Kurtoglu, Meltem Ayli, Tülin Firatli Tuglular, Fevzi Altuntas, Levent Undar","doi":"10.4274/tjh.galenos.2024.2024.0450","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0450","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 a poor prognosis.</p><p><strong>Materials and methods: </strong>Data from 53 patients who were diagnosed with pPCL between 2011-2020 and who used at least one proteasome inhibitor (PI) and/or immunomodulatory (IMID) agent were analyzed retrospectively. Depending on the year of pPCL diagnosis, 20% leukocytes or ≥2×109/L plasma cells in the peripheral blood were used.</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 in 31 (58.5%) patients, and PI and IMID were used simultaneously in 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 (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 of 15 months. Only 7 (13.2%) of the patients were alive at the last follow-up visit. Those with higher beta-2 microglobulin levels and ISS stage 3 and nontransplant patients receiving first-line treatment had shorter OS (p=0.005, p=0.02 and p=0.008, respectively). Otherwise, 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 our study, as in previous similar studies, we could not see the increased survival trend in pPCL which is observed in MM. New studies are needed for pPCL patients, which is likely to increase with the new diagnostic criteria, based on current agents and information in MM.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","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":"https://doi.org/10.4274/tjh.galenos.2024.2024.0339","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"GATA3 Immunohistochemical Staining in Classical Hodgkin Lymphoma and its Diagnostic Utility in Differential Diagnosis.","authors":"Ali Yılmaz Altay, Begüm Yeni Erdem, Gülçin Yegen","doi":"10.4274/tjh.galenos.2024.2024.0237","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0237","url":null,"abstract":"<p><strong>Objective: </strong>Classical Hodgkin lymphoma (CHL) is a common lymphoid neoplasm with a wide range of differential diagnosis. Although it has a specific immunophenotype, aberrant expression of antigens can cause problems at its diagnosis. In this study we evaluated the usefulness of GATA3 in differential diagnosis of classical Hodgkin lymphoma.</p><p><strong>Material and method: </strong>One hundred CHL cases and a control group of 106 lymphoma cases, which include anaplastic large cell lymphoma (ALK (+) and (-)), EBV (+) large B cell lymphoma, T-cell/ histiocyte rich B cell lymphoma, primary mediastinal large B cell lymphoma, nodular lymphocyte predominant Hodgkin lymphoma and mediastinal grey zone lymphoma, were included in the study. GATA3 immunohistochemistry were applied to all cases and its nuclear expression was accepted as positive. Expression status of GATA3 was compared in between the CHL and the control group, as well as among each lymphoma subtype. In addition, whether the biopsy type effects its diagnostic performance is assessed. In CHLs relationship with EBV status and GATA3 expression is evaluated.</p><p><strong>Results: </strong>GATA3 expression was significantly higher in CHL cases compared to the control group (p<0,001). When compared with the individual subgroups GATA3 is still found to be useful in differential diagnosis except for ALK (-) ALCL (p=0,678) and mediastinal gray zone lymphomas (p=0,327). GATA3 expression is significantly higher in EBV (-) CHLs (p=0,02). In core needle biopsies its diagnostic performance is limited (p=0,178).</p><p><strong>Conclusions: </strong>GATA3 is a useful marker for differentiating CHL from B-cell non-Hodgkin lymphomas but its efficiency is limited in ALK (-) ALCL and mediastinal grey zone lymphomas. Due to the heterogeneous reaction diagnostic value is limited in core needle biopsies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142547743","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":"The Role of the Hemostasis and Thrombosis Unit in the Management of the Patients with Acquired Hemophilia.","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":"https://doi.org/10.4274/tjh.galenos.2024.2024.0230","url":null,"abstract":"<p><strong>Objective: </strong>Acquired hemophilia A (AHA) is a rare autoimmune disease characterised by the presence of autoantibodies against coagulation factor VIII (FVIII), leading to spontaneous haemorrhage 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, along with specific case reports.</p><p><strong>Materials and methods: </strong>Diagnosis and treatment occurred between 2005 and 2023. The median age at diagnosis was 67.8 years (range 15-93). Among the 41 patients, 10 (24%) were idiopathic, 4 (10%) were postpartum, 18 (44%) had autoimmune diseases, and 9 were diagnosed with cancer.</p><p><strong>Results: </strong>Diagnostic delay exceeded 30 days in 15 out of the 41 cases (36.5%). A total of 38 out 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 in 100% of patients, and no patient died.</p><p><strong>Conclusion: </strong>Haemostatic 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. The absence of mortality is likely attributable to the management of the disease within a specialised Haemostasis and Thrombosis Unit, which offers a clinical ward, a specialised laboratory, and a dedicated ambulatory service. The Italian Society for the Study of Haemostasis and Thrombosis (SISET) is working to secure recognition for this essential role in every hospital.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","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, Multi-center, Retrospective BHOLISTIC 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 Beşişik, Can Boğa, Guray Saydam, Zhongwen Huang, Jan Pinchevsky, Burhan Ferhanoğlu","doi":"10.4274/tjh.galenos.2024.2024.0181","DOIUrl":"https://doi.org/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 relapsed/refractory HL (RRHL) from January 2010-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, cisplatin (ESHAP) in Saudi Arabia (78.3%) and dexamethasone, cytarabine, cisplatin (DHAP) 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>Conclusions: </strong>This study showed that the clinical outcomes in Türkiye and Saudi Arabia were generally comparable with 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. Additionally, 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":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unexpected Discoveries: Eosinophilia Unmasked by Splenic Microfilariasis in a Young Woman.","authors":"Bilal Kazi, Sweety Shinde, Imaan Rumani, Marzooka Nazim Chishti Kazi","doi":"10.4274/tjh.galenos.2024.2024.0299","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2024.2024.0299","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142475834","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":"Dilemma in 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":"https://doi.org/10.4274/tjh.galenos.2024.2024.0280","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2024-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}