Turkish Journal of Hematology最新文献

{"title":"Unexpected Discoveries: Eosinophilia Masking 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":"10.4274/tjh.galenos.2024.2024.0299","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"279-280"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628757/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Secondary Solid Cancers in Patients with Philadelphia Chromosome-Negative Myeloproliferative Neoplasms: A Multicenter Study","authors":"Fehmi Hindilerden, Özge Nuran Akay, Elif Aksoy, Aynur Dağlar-Aday, Emine Gültürk, Meliha Nalçacı, İpek Yönal-Hindilerden","doi":"10.4274/tjh.galenos.2024.2024.0199","DOIUrl":"10.4274/tjh.galenos.2024.2024.0199","url":null,"abstract":"<p><strong>Objective: </strong>We investigated the occurrence and characteristics of secondary solid cancers (SSCs) in patients with Philadelphia chromosome-negative myeloproliferative neoplasms (Ph- MPNs) from Türkiye. We identified the potential risk factors for SSC development, including the impact of cytoreductive therapies, and we assessed the influence of SSC on patient survival.</p><p><strong>Materials and methods: </strong>A total of 1013 Ph- MPN patients diagnosed between 1995 and 2022 were retrospectively analyzed. Data related to demographics, clinical and laboratory parameters, SSC development, cytoreductive therapy exposure, and survival outcomes were collected. Statistical analyses were performed using IBM SPSS Statistics 26.0.</p><p><strong>Results: </strong>Of the analyzed Ph- MPN patients, 6.6% developed SSC, with carcinoma being the most common type. Older age at the time of Ph- MPN diagnosis and male sex were associated with SSC occurrence. Ph- MPN patients diagnosed with SSC and patients with no diagnosis of SSC showed no significant difference in complete blood count results, spleen size, Ph- MPN diagnostic groups, or driver mutation frequencies. However, patients with SSC had a higher frequency of arterial thrombosis and a tendency towards an increased rate of total thrombosis (p=0.030 and p=0.069, respectively). In multivariate analysis, arterial thrombosis was the sole independent risk factor and interferon (IFN)-based therapy was the sole protective factor for SSC development. Median overall survival (OS) did not differ between patients with and without SSC except for polycythemia vera patients with SSC, who had shorter OS (175±15 versus 321±26 months, respectively; p=0.005).</p><p><strong>Conclusion: </strong>This study highlights the prevalence and characteristics of SSCs in Turkish patients diagnosed with Ph- MPNs. Arterial thrombosis was associated with increased SSC risk while IFN-based therapy offered potential protection from SSC. Screening for SSC in Ph- MPN patients with arterial thrombosis may be valuable. These findings emphasize the importance of malignancy screening in Ph- MPN patients, especially in high-risk subgroups, and call for further research to elucidate the underlying mechanisms and optimize treatment strategies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"246-255"},"PeriodicalIF":1.5,"publicationDate":"2024-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11628762/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141903031","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":"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":"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":"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":" ","pages":""},"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}

{"title":"Epstein-Barr Virus-Induced Warm Autoimmune Hemolytic Anemia","authors":"Kazi Bilal, Chakrapani Anupam, Ramasubban Suresh, Das Sudipta, Kazi Chishti Marzooka","doi":"10.4274/tjh.galenos.2024.2024.0136","DOIUrl":"10.4274/tjh.galenos.2024.2024.0136","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"209-211"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589368/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141474680","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":"Anti-CD22 Calicheamicin-Inotuzumab Ozogamicin Combined with Venetoclax + Azacitidine in the Treatment of Mixed-Phenotype Acute Leukemia: A Case Report","authors":"Kongfei Li, Yuxiao Wang, Renzhi Pei, Ying Lu, Junjie Cao, Xianxu Zhuang, Jiaying Lian, Bibo Zhang","doi":"10.4274/tjh.galenos.2024.2024.0044","DOIUrl":"10.4274/tjh.galenos.2024.2024.0044","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"202-204"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589369/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141793614","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":"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":" ","pages":"133-140"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589373/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"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":" ","pages":"188-189"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589367/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cleaved Leukemic Blast Cells, Vacuolation, and Pseudopodia-like Cytoplasmic Projections in Acute Myeloid Leukemia with <i>TLS::ERG</i>","authors":"Xingqin Huang, Linglin Jiang, Ting Li, Mei Yang","doi":"10.4274/tjh.galenos.2024.2023.0386","DOIUrl":"10.4274/tjh.galenos.2024.2023.0386","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"192-193"},"PeriodicalIF":1.5,"publicationDate":"2024-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11589370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139576788","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}