Turkish Journal of Hematology最新文献

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Retrospective Evaluation of Clinical And Follow-Up Outcomes in Primary Cutaneous CD30+ Lymphoproliferative Disorders.
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-03-06 DOI: 10.4274/tjh.galenos.2025.2025.0045
Hatice Şanlı, Ahmet Taha Aydemir, İncilay Kalay Yıldızhan, Aylin Heper, Işınsu Kuzu, Ayça Kırmızı, Ayşenur Botsalı, Bengü Nisa Akay
{"title":"Retrospective Evaluation of Clinical And Follow-Up Outcomes in Primary Cutaneous CD30+ Lymphoproliferative Disorders.","authors":"Hatice Şanlı, Ahmet Taha Aydemir, İncilay Kalay Yıldızhan, Aylin Heper, Işınsu Kuzu, Ayça Kırmızı, Ayşenur Botsalı, Bengü Nisa Akay","doi":"10.4274/tjh.galenos.2025.2025.0045","DOIUrl":"https://doi.org/10.4274/tjh.galenos.2025.2025.0045","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.5,"publicationDate":"2025-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143567686","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}
引用次数: 0
Images of Temporomandibular Joint Arthropathy in Patient with Severe Hemophilia B with Inhibitor and Patient with Type III von Willebrand Disease with Inhibitor 一名严重血友病 B 型患者(含抑制剂)和一名 III 型 Von Willebrand 患者(含抑制剂)的颞下颌关节关节病图像。
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2024-09-25 DOI: 10.4274/tjh.galenos.2024.2024.0342
Selda Yenel, Dilek Aynur Çankal, Zühre Akarslan, Merve Yazol, Zühre Kaya
{"title":"Images of Temporomandibular Joint Arthropathy in Patient with Severe Hemophilia B with Inhibitor and Patient with Type III von Willebrand Disease with Inhibitor","authors":"Selda Yenel, Dilek Aynur Çankal, Zühre Akarslan, Merve Yazol, Zühre Kaya","doi":"10.4274/tjh.galenos.2024.2024.0342","DOIUrl":"10.4274/tjh.galenos.2024.2024.0342","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"59-60"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869152/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Late Effects Following Hematopoietic Stem Cell Transplantation Among Childhood Transplant Survivors with Fanconi Anemia 患有范可尼贫血症的儿童移植幸存者在造血干细胞移植后的晚期影响
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2024-11-08 DOI: 10.4274/tjh.galenos.2024.2024.0189
Cansu Özkoçer, Fatma Visal Okur, Hüseyin Demirbilek, Burak Altıntaş, Nevin Çetin, Barış Kuşkonmaz, Bora Gülhan, Hayrettin Hakan Aykan, Hülya Demir, Deniz Doğru Ersöz, Uğur Canpolat, Hasan Serkan Doğan, Elmas Nazlı Gönç, Hatice Yasemin Balaban, Gürkan Bozdağ, Şule Ünal, Şevkiye Selin Aytaç Eyüpoğlu, Rezan Topaloğlu, Zeynep Alev Özön, Fatma Gümrük, Duygu Uçkan Çetinkaya
{"title":"Late Effects Following Hematopoietic Stem Cell Transplantation Among Childhood Transplant Survivors with Fanconi Anemia","authors":"Cansu Özkoçer, Fatma Visal Okur, Hüseyin Demirbilek, Burak Altıntaş, Nevin Çetin, Barış Kuşkonmaz, Bora Gülhan, Hayrettin Hakan Aykan, Hülya Demir, Deniz Doğru Ersöz, Uğur Canpolat, Hasan Serkan Doğan, Elmas Nazlı Gönç, Hatice Yasemin Balaban, Gürkan Bozdağ, Şule Ünal, Şevkiye Selin Aytaç Eyüpoğlu, Rezan Topaloğlu, Zeynep Alev Özön, Fatma Gümrük, Duygu Uçkan Çetinkaya","doi":"10.4274/tjh.galenos.2024.2024.0189","DOIUrl":"10.4274/tjh.galenos.2024.2024.0189","url":null,"abstract":"<p><strong>Objective: </strong>This study was planned to evaluate long-term post-transplant complications in patients who underwent transplantation with the diagnosis of Fanconi anemia (FA) in childhood in our bone marrow transplantation unit and who were still being followed. It was predicted that the results would show the critical importance of determining disease-specific post-transplant long-term follow-up plans and putting them into practice in terms of early detection of complications and improving the survival rates and quality of life of FA patients.</p><p><strong>Materials and methods: </strong>In this single-center, cross-sectional study, according to current recommendations, we analyzed the long-term outcomes of 36 patients with FA with a median age of 18.1 years (range: 6.1-36 years, male/female ratio: 24/12) who underwent HSCT in the Pediatric Bone Marrow Transplantation Unit between 1995 and 2019 and survived at least 1 year following the transplantation.</p><p><strong>Results: </strong>The median long-term follow-up time was 8 years (range: 1-25 years). Gonadal dysfunction was detected in approximately 35% of our patients; more specifically, 31% of the patients had hypergonadotropic hypogonadism and 4% had hypogonadotropic hypogonadism. When the patients were evaluated for growth impairment, 7 of 12 patients who had reached their final adult heights and 12 of 21 patients who had not yet completed their growth had height standard deviation (SD) scores below -2 SDs. Three patients (9%) developed subclinical hypothyroidism, 2 (6%) had overt hypothyroidism, and 1 (3%) had central hypothyroidism. Although none of our patients fully met the criteria for metabolic syndrome, 23% had insulin resistance and 39% had dyslipidemia. Evaluation of organ dysfunctions revealed that nearly 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 1 patient.</p><p><strong>Conclusion: </strong>A risk-defined multidisciplinary approach for the 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":"15-24"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869150/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Identification of TRAPPC4 as a Key Autoantigen in Immune-Related Pancytopenia: Epitope Characterization and Immune Activation Mechanisms TRAPPC4作为免疫相关性全细胞减少症关键自身抗原的鉴定:表位表征和免疫激活机制。
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2025-01-17 DOI: 10.4274/tjh.galenos.2025.2024.0365
Shanfeng Hao, Yang Zhang, Na Xiao, Zonghong Shao
{"title":"Identification of TRAPPC4 as a Key Autoantigen in Immune-Related Pancytopenia: Epitope Characterization and Immune Activation Mechanisms","authors":"Shanfeng Hao, Yang Zhang, Na Xiao, Zonghong Shao","doi":"10.4274/tjh.galenos.2025.2024.0365","DOIUrl":"10.4274/tjh.galenos.2025.2024.0365","url":null,"abstract":"<p><strong>Objective: </strong>Immune-related pancytopenia (IRP) is characterized by autoantibody-mediated destruction or suppression of bone marrow cells, leading to pancytopenia. This study aimed to explore the role of trafficking protein particle complex subunit 4 (TRAPPC4) as a key autoantigen in IRP, including epitope identification and immune activation mechanisms.</p><p><strong>Materials and methods: </strong>A total of 90 participants were included in the study, divided into four groups: 30 newly diagnosed IRP patients, 25 patients with IRP in remission, 20 patients with other hematological conditions (severe aplastic anemia [SAA] and myelodysplastic syndrome [MDS]) as a patient control group, and 15 healthy individuals as a healthy control group. TRAPPC4 was identified using affinity screening with a phage-display random peptide library and confirmed with ELISPOT and epitope prediction software. TRAPPC4 expression in bone marrow cells and serum antibody titers was assessed via flow cytometry, ELISA assay, and real-time polymerase chain reaction. Immune cell profiling of peripheral blood mononuclear cells was conducted using flow cytometry.</p><p><strong>Results: </strong>TRAPPC4 was overexpressed in the CD34+ bone marrow hematopoietic progenitor cells of newly diagnosed IRP patients compared to patients in remission, the patient control group (SAA and MDS), and the healthy control group, with no significant differences observed for CD15+ granulocytes or CD235a+ nucleated red blood cells. The epitope peptide YTADGKEVLEYLG activated Th2 cells, as confirmed by ELISPOT. Newly diagnosed IRP patients exhibited elevated TRAPPC4 mRNA and protein levels in bone marrow mononuclear cells and higher serum antibody titers compared to controls. Immune profiling revealed increased CD19+ and CD5+CD19+ B lymphocytes in IRP patients.</p><p><strong>Conclusion: </strong>TRAPPC4 was found to be a key autoantigen in IRP along with CD34+ cells as primary targets of autoantibody attacks. The identification of TRAPPC4 and its epitope provides insights into IRP pathogenesis and suggests potential diagnostic and therapeutic strategies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"33-46"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143012447","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}
引用次数: 0
Aseptic Abscess Syndrome: A Unique Case of Splenic Involvement and Systemic Inflammation. 无菌性脓肿综合征:脾脏受累和全身炎症的独特病例
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 DOI: 10.4274/tjh.galenos.2025.2025.0010
Betül Yılmaz, Merve Güzel Dirim, Burak Gültekin, Naci Şenkal, Alpay Medetalibeyoğlu, Gülçin Yegen, Murat Köse, Arif Atahan Çağatay
{"title":"Aseptic Abscess Syndrome: A Unique Case of Splenic Involvement and Systemic Inflammation.","authors":"Betül Yılmaz, Merve Güzel Dirim, Burak Gültekin, Naci Şenkal, Alpay Medetalibeyoğlu, Gülçin Yegen, Murat Köse, Arif Atahan Çağatay","doi":"10.4274/tjh.galenos.2025.2025.0010","DOIUrl":"10.4274/tjh.galenos.2025.2025.0010","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":"42 1","pages":"72-73"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869141/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143524552","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}
引用次数: 0
GATA3 Immunohistochemical Staining in Classical Hodgkin Lymphoma and Its Diagnostic Utility in Differential Diagnosis 经典霍奇金淋巴瘤中的 GATA3 免疫组织化学染色及其在鉴别诊断中的作用
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2024-10-31 DOI: 10.4274/tjh.galenos.2024.2024.0237
Ali Yılmaz Altay, Begüm Yeni Erdem, Gülçin Yegen
{"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":"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 diagnoses. 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 the differential diagnosis of CHL.</p><p><strong>Materials and methods: </strong>One hundred cases of CHL and a control group of 106 lymphoma cases, which included anaplastic large-cell lymphoma both positive and negative for anaplastic lymphoma kinase (ALK), Epstein-Barr virus (EBV)-positive large B-cell lymphoma, T-cell/histiocyte-rich B-cell lymphoma, primary mediastinal large B-cell lymphoma, nodular lymphocyte-predominant Hodgkin lymphoma, and mediastinal gray-zone lymphoma, were included in the study. GATA3 immunohistochemistry was applied to all cases and nuclear expression was accepted as positive. Expression status of GATA3 was compared between the CHL group and the control group, as well as among each lymphoma subtype. In addition, whether the biopsy type affected diagnostic performance was assessed. For CHL, the relationship with EBV status and GATA3 expression was evaluated.</p><p><strong>Results: </strong>GATA3 expression was significantly higher in CHL cases compared to the control group (p<0.001). When compared among individual subgroups, GATA3 was found to be useful in the differential diagnosis of all except for ALK-negative anaplastic large-cell lymphoma (p=0.678) and mediastinal gray-zone lymphoma (p=0.327). GATA3 expression was significantly higher in EBV-negative CHL (p=0.02). In core-needle biopsies, the diagnostic performance was limited (p=0.178).</p><p><strong>Conclusion: </strong>GATA3 is a useful marker for differentiating CHL from B-cell non-Hodgkin lymphomas but its efficiency is limited in ALK-negative anaplastic large-cell lymphoma and mediastinal gray-zone lymphoma. Due to heterogeneous reactions, its diagnostic value is limited in core-needle biopsies.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"9-14"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869154/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Navigating Hope and Complexity: Turkish Parents’ Experiences with Savior Siblings
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2025-01-23 DOI: 10.4274/tjh.galenos.2025.2024.0419
İbrahim Eker, Hamide Nur Çevik Özdemir, Fırat Yılmaz, Akif Yeşilipek, Alphan Küpesiz, Vedat Uygun, Gülsün Karasu, Funda Tayfun Küpesiz, Orhan Gürsel, Barış Kuşkonmaz, Serap Aksoylar, Fatma Visal Okur, Gülcihan Özek, Musa Karakükcü, Başak Adaklı Aksoy, Özlem Tüfekçi, Zühre Kaya, Barış Malbora, Ahmet Emin Kürekçi, Ali Bülent Antmen
{"title":"Navigating Hope and Complexity: Turkish Parents’ Experiences with Savior Siblings","authors":"İbrahim Eker, Hamide Nur Çevik Özdemir, Fırat Yılmaz, Akif Yeşilipek, Alphan Küpesiz, Vedat Uygun, Gülsün Karasu, Funda Tayfun Küpesiz, Orhan Gürsel, Barış Kuşkonmaz, Serap Aksoylar, Fatma Visal Okur, Gülcihan Özek, Musa Karakükcü, Başak Adaklı Aksoy, Özlem Tüfekçi, Zühre Kaya, Barış Malbora, Ahmet Emin Kürekçi, Ali Bülent Antmen","doi":"10.4274/tjh.galenos.2025.2024.0419","DOIUrl":"10.4274/tjh.galenos.2025.2024.0419","url":null,"abstract":"<p><strong>Objective: </strong>Preimplantation genetic diagnosis (PGD) with human leukocyte antigen (HLA) typing represents a significant advancement in treating inherited hematological disorders, particularly thalassemia major. This technology enables the birth of healthy children who can serve as compatible stem cell donors for their affected siblings. Türkiye is a world leader in both PGD+HLA typing technology and hematopoietic stem cell transplantation (HSCT) from savior siblings born through PGD+HLA typing. This study investigated the experiences of Turkish parents who underwent successful savior sibling procedures using PGD+HLA typing and then successful HSCT from the savior sibling for the treatment of the child with thalassemia major. We aimed to understand the medical, psychological, and sociocultural dimensions of this complex process within the Turkish healthcare context.</p><p><strong>Materials and methods: </strong>A qualitative study was undertaken using a descriptive phenomenological approach. In-depth interviews were conducted with parents from 16 families who had successfully completed PGD+HLA matching and subsequent stem cell transplantation processes from the savior sibling to the child with thalassemia. Data were analyzed using Colaizzi’s seven-step method and MAXQDA 20.0 software.</p><p><strong>Results: </strong>The analysis revealed six main themes: disease stage, treatment, recovery process, social/family, support systems, and recommendations. Parents reported significant emotional challenges but demonstrated unexpected resilience. Religious and cultural factors played nuanced roles, with most parents viewing the process as compatible with their beliefs. Economic burdens, prolonged hospitalizations, and geographical access to treatment centers emerged as key challenges. Extended family support and professional healthcare guidance were identified as crucial support mechanisms.</p><p><strong>Conclusion: </strong>This study highlights the complex interplay between advanced medical technologies and traditional values in Turkish society. The findings emphasize the need for comprehensive and culturally sensitive support systems and long-term follow-up for families. The results suggest the value of implementing multidisciplinary care teams and developing specialized support programs for families undergoing savior sibling procedures.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"47-55"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869153/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024921","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}
引用次数: 0
IRF4 rs12203592 Risk Variant Shows Effect in European But Not Non-European and Admixed Myelodysplastic Syndrome Patients
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2025-01-29 DOI: 10.4274/tjh.galenos.2025.2024.0386
Jonas Nogueira Ferreira Maciel Gusmão, Alessandro Cavalcante Chaves, João Vitor Caetano Goes, Ronald Feitosa Pinheiro, Howard Lopes Ribeiro Júnior
{"title":"<i>IRF4</i> rs12203592 Risk Variant Shows Effect in European But Not Non-European and Admixed Myelodysplastic Syndrome Patients","authors":"Jonas Nogueira Ferreira Maciel Gusmão, Alessandro Cavalcante Chaves, João Vitor Caetano Goes, Ronald Feitosa Pinheiro, Howard Lopes Ribeiro Júnior","doi":"10.4274/tjh.galenos.2025.2024.0386","DOIUrl":"10.4274/tjh.galenos.2025.2024.0386","url":null,"abstract":"","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"65-68"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869148/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143060856","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}
引用次数: 0
TAFRO Syndrome Without Pathology Supporting Castleman Disease: To Be Treated as Idiopathic Multicentric Castleman Disease-TAFRO or a Distinct Disease Entity?
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2025-02-12 DOI: 10.4274/tjh.galenos.2025.2024.0420
Si-Yuan Li, Yu-Han Gao, Yue Dang, Lu Zhang, Jian Li
{"title":"TAFRO Syndrome Without Pathology Supporting Castleman Disease: To Be Treated as Idiopathic Multicentric Castleman Disease-TAFRO or a Distinct Disease Entity?","authors":"Si-Yuan Li, Yu-Han Gao, Yue Dang, Lu Zhang, Jian Li","doi":"10.4274/tjh.galenos.2025.2024.0420","DOIUrl":"10.4274/tjh.galenos.2025.2024.0420","url":null,"abstract":"<p><strong>Objective: </strong>TAFRO syndrome, entailing thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly, was previously considered a subtype of idiopathic multicentric Castleman disease (iMCD-TAFRO), with the diagnosis requiring pathology supporting Castleman disease. However, lymph node biopsies may be difficult for TAFRO patients (TAFRO without pathological evidence: TAFRO-w/op-iMCD), and sometimes these biopsies do not confirm iMCD (TAFRO-w/o-iMCD). We aimed to compare the clinical features and prognosis of TAFRO subgroups.</p><p><strong>Materials and methods: </strong>We retrospectively analyzed the cases of 50 iMCD-TAFRO and 11 TAFRO-w/o-iMCD patients treated from May 2015 to April 2024.</p><p><strong>Results: </strong>The groups showed no significant differences in clinical presentation or laboratory data. Both groups of patients were treated with iMCD-targeted strategies addressing cytokine storms. With a median follow-up of 21.4 (range: 0.5-107.0) months, there were no significant differences between iMCD-TAFRO and TAFRO-w/o-iMCD patients in 3-month response rate (72.1% vs. 88.9%, p=0.525), 6-month response rate (70.0% vs. 83.3%, p=0.849), or best overall response rate (77.6% vs. 90.0%, p=0.645). The estimated 3-year progression-free survival rate (65.8% vs. 90.0%, log-rank p=0.163) and the estimated 3-year overall survival rate (77.0% vs. 100%, log-rank p=0.145) were also not significantly different. Cox univariate analysis showed that decreased estimated glomerular filtration rate (<60 mL/min/1.73 m<sup>2</sup>) was associated with an increased risk of disease progression (hazard ratio: 4.133, 95% confidence interval: 1.561-10.940, p=0.004).</p><p><strong>Conclusion: </strong>iMCD-TAFRO and TAFRO-w/o-iMCD could be considered overlapping entities and these patients should be treated promptly, targeting cytokine storms with similar strategies for each group of patients.</p>","PeriodicalId":23362,"journal":{"name":"Turkish Journal of Hematology","volume":" ","pages":"1-8"},"PeriodicalIF":1.5,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11869139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399360","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}
引用次数: 0
Multigene Panel Testing Reveals Novel Variants in Hereditary Spherocytosis Patients in Türkiye 多基因面板检测揭示了遗传性球形红细胞增多症患者的新变异。
IF 1.5 4区 医学
Turkish Journal of Hematology Pub Date : 2025-02-28 Epub Date: 2025-01-06 DOI: 10.4274/tjh.galenos.2025.2024.0270
Ömer Doğru, Ceren Alavanda, Şenol Demir, Ahmet Koç, Pınar Ata
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