Hematology, Transfusion and Cell Therapy最新文献

{"title":"INDUCTION CHEMOTHERAPY IN ADVANCED HEAD AND NECK SQUAMOUS CELL CARCINOMA: A REAL-WORLD DATA STUDY","authors":"Matheus Yung Perin, Vivian Naomi Horita, Daniel Naves Araújo Teixeira, Joyce Gruenwaldt, Eduardo Baldon Pereira, Carlos Takahiro Chone, Gustavo Jacob Lourenço, Ligia Traldi Macedo, Carmen Silvia Passos Lima","doi":"10.1016/j.htct.2025.103773","DOIUrl":"10.1016/j.htct.2025.103773","url":null,"abstract":"<div><h3>Introduction/Justification</h3><div>Approximately 60% of HNSCC patients are diagnosed at a locally or locoregionally advanced stage. Patients with locally or locoregionally advanced stage and not amenable to surgical resection receive chemoradiotherapy (CTRT) as definitive treatment, or induction chemotherapy (ICT) followed or not by CTR. In the last scenery, docetaxel plus cisplatin (TP) and docetaxel plus cisplatin plus 5-fluorouracil (TPF) followed by CTRT were first described as effective ICTs regimens with acceptable safety profile. Despite the superiority of TPF over TP in response rate, loco-regional control, and survival of patients with advanced HNSCC, unequivocal disadvantages have been attributed to the regimen, as grade 3 or above adverse events, and the need of infusion devices or inpatient beds for continuous 5-fluorouracil infusion, which clearly increases the costs of treatment.</div></div><div><h3>Objectives</h3><div>The current study aimed to analyze patients with locoregionally advanced HNSCC treated with TPF or TP followed by CTRT at the General Hospital of the University of Campinas, with the purpose of developing an ICT protocol applicable to services with limited resources.</div></div><div><h3>Materials and Methods</h3><div>Patients with HNSCC at stage III or IVA-B (T4 and/or N2b, N2c or N3) treated with ICT using TPF or TP followed by CTRT from January 14 th, 2015, to November 24 th, 2021, were included in the study. The choice between TPF and TP as induction chemotherapy (ICT) was based on the clinical judgment of the responsible oncologist, considering patient-specific factors such as performance status, comorbidities, and tolerance to intensive regimens. Additionally, the availability of a hospital bed for the continuous intravenous infusion of 5-fluorouracil was a practical determinant. Toxicity, response rate, and event-free survival (EFS) and overall survival (OS) were evaluated in patients of both groups. Event-free survival (EFS) and overall survival (OS) were assessed using the Kaplan-Meier curves and the log-rank test. The impact of clinicopathological characteristics on patients’ survival was assessed through univariate and multivariate Cox regression.</div></div><div><h3>Results</h3><div>Eighty-seven patients with HNSCC were treated with ICT, being 38 with TPF and 49 with TP. An excess of ECOG 0 or 1 was seen in TPF group and an excess of males in TP group, but no significant differences in age, smoking and alcohol intake, body mass index, tumor location, grade and TNM stage, toxicities grade 3 or above, treatment response, and cycles interval, were seen in patients treated with TPF and TP. The median follow-up time was 22.6 months (range: 1.2 to 93.8). The two-year and five-year EFS rates of patients of the total group were 33.8% and 25.3%, respectively. ICT regimens did not alter response to ICT, and patients’ EFS and OS. Cox multivariate analysis identified stable or progressive disease (HR: 5.56) a","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103773"},"PeriodicalIF":1.8,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143828166","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ANTIPROLIFERATIVE ACTIVITIES IN VITRO OVER SQUAMOUS CELL CARCINOMAS OF A PALLADIUM(II) COMPLEX WITH AMANTADINE","authors":"Pedro Corbi, Laura Barros Silva, Fernanda Van Petten V. Azevedo, Gilberto C. Franchi Jr, Carmen Silvia P. Lima","doi":"10.1016/j.htct.2025.103777","DOIUrl":"10.1016/j.htct.2025.103777","url":null,"abstract":"<div><h3>Introduction/Justification</h3><div>Metal-based drugs have been used in diagnosis and treatment of different types of cancer since the discovery of cisplatin's antineoplastic properties in the 1960’s. Second-generation drugs based on the platinum(II) complex cisplatin, such as carboplatin and oxaliplatin, were developed and used for cancer treatment worldwide. However, platinum(II) drugs typically cause side effects, such as nephrotoxicity, neurotoxicity and myelosuppression, which motivates the search for new drug candidates. Since the platinum(II) and palladium(II) ions have similar characteristics and form analogous compounds, palladium(II) complexes have been also studied as potential anticancer agents. Recently, a new palladium(II) drug named padeliporfin (Tookad®Soluble) has entered the clinic for the treatment of low-risk prostate cancer, which further motivates the investigation of palladium(II) complexes as potential antineoplastic drugs. Adamantanes are a class of organic compounds that consist of a single diamond-like carbon cage. The functionalization of adamantane with an amine group lead to amantadine, which has been used in the clinic as an antiviral and anti-Parkinson drug and has also been evaluated for its anticancer activity.</div></div><div><h3>Objectives</h3><div>In this study, we report for the first time the antiproliferative studies of a palladium(II) complex with amantadine (Pd-atd) over squamous cell carcinomas</div></div><div><h3>Materials and Methods</h3><div>The Pd-atd complex was prepared following the literature protocol. Briefly, the complex was prepared by the reaction of Li2PdCl4 with amantadine hydrochloride in methanol under stirring and at room temperature. The yellowish solid obtained was collected by filtration, washed with methanol and dried. Yield 72%. The [PdCl2(C10H17N)2] composition was confirmed by chemical and spectroscopic analyses. Squamous cell carcinoma of tongue (SCC-4 and SCC-25) and of hypopharynx (Fadu), and a non-tumoral cell line (HaCat, immortalized keratinocyte) were used in this study. Cells were cultivated following the methodology previously described in the literature. Cell viability was determined by dose-response curves obtained from an MTT assay measuring the absorbance after 48h.</div></div><div><h3>Results</h3><div>The Pd-atd complex inhibited proliferation of SCC-4 cells with an IC50 of 1.87 µM and it was non-toxic to HaCat cells. Cisplatin, a standard drug, presented an IC50 of 7.02 µM and it was less selective toward HaCat cells in the same experimental conditions.</div></div><div><h3>Conclusion</h3><div>The promising results of the antiproliferative activities of the Pd-atd complex over SCC-4 cells warrant for additional studies about the potential of application of the complex as an antiproliferative agent for the treatment of squamous cell carcinomas.</div></div><div><h3>Acknowledgements</h3><div>This study was supported by grants from the Brazilian Agencies FAPESP ","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103777"},"PeriodicalIF":1.8,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143828170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"PROTOCOL OF A RANDOMIZED PILOT STUDY ON SURVIVAL IN NEWLY DIAGNOSED GLIOBLASTOMA PATIENTS UNDERGOING CHEMORADIATION VERSUS COMBINED CHEMORADIATION WITH INTRANASAL PERILLYL ALCOHOL","authors":"DANIELA CARNEIRO DE Lima , Alex H. Schöntal , Clóvis Orlando Pereira da Fonseca , Fabiano Reis , Carmen Silva Passos Lima , Mary Ann Foglio","doi":"10.1016/j.htct.2025.103776","DOIUrl":"10.1016/j.htct.2025.103776","url":null,"abstract":"<div><h3>Introduction/Justification</h3><div>Glioblastoma multiforme (GBM) is a highly aggressive type of brain tumor and remains one of the most challenging cancers to treat. This tumor is characterized by rapid progression and unfavorable prognosis. After undergoing surgery, radiotherapy (RT), and chemotherapy (CT) with temozolomide (TMZ), patients generally exhibit low survival rates. Perillyl alcohol (POH) is a hydroxylated monoterpene with antitumor, anti-angiogenic, and pro-apoptotic properties, inhibiting RAS oncogene-mediated signaling. In vitro and in vivo studies showed POH's cytotoxicity to both TMZ-resistant and sensitive cells, and its effectiveness as a radiosensitizer in malignant glioma cell lines. In phase I/II trials with oral POH in advanced, refractory cancer patients, nausea and other gastrointestinal toxicities led to study discontinuation. Currently, POH is being studied as an inhaled anticancer agent, showing no toxicity and promising activity with increased survival in patients with recurrent gliomas, however, these studies were not randomized, and to date, these investigations have been conducted exclusively in patients with recurrent gliomas.</div></div><div><h3>Objectives</h3><div>This study presents a protocol from the University of Campinas, developed by experts across multiple fields, to evaluate the effects of intranasal POH in GBM patients.</div></div><div><h3>Materials and Methods</h3><div>Patient's will be recruited from the Oncology Service at the General Hospital of the University of Campinas (UNICAMP) after tumor resection, with a total of 40 participants. Adult individuals of any gender will be included. Through randomization, participants will be randomly assigned to two groups: the control group (RT+CT) and the intervention group (RT+CT+ POH inhalations). Block randomization of four patients will ensure balance between groups during recruitment. The randomization sequence was generated at www.randomization.com. The control group will undergo RT and CT with TMZ: 75 mg/m² of TMZ daily for 6 weeks during RT (2 Gy/day for 5 days a week, totaling 60 Gy), followed by 150 or 200 mg/m² of TMZ for 5 days per cycle in 28-day cycles for 6 cycles. The participants in the intervention group will receive the same treatment plus 0.3% POH inhalations (55 mg in 3 mL of water, 4 times daily, with 6-hour intervals). They will undergo 6 weeks of RT + TMZ + POH, followed by 6 cycles of TMZ + POH (5 days of TMZ + POH, followed by 23 days of POH only). Cranial MRIs will be analyzed by an expert neuroradiologist using T2/FLAIR signal intensity with perfusion, without knowledge of patient group allocation. MRIs are part of routine treatment, performed every 3-4 months in the first year, and response will be assessed using MacDonald and RANO criteria for high-grade gliomas. Toxicity assessment of standard treatment with POH will follow the NCI Common Terminology Criteria, version 5.0. Progression-free survival will be compared betw","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 ","pages":"Article 103776"},"PeriodicalIF":1.8,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143828169","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis and treatment of chronic lymphocytic leukemia: 2025 recommendations of the Brazilian Group of Chronic Lymphocytic Leukemia of the Brazilian Association of Hematology and Hemotherapy (ABHH)","authors":"Carlos Sérgio Chiattone ,&nbsp;Fernanda de Morais Marques ,&nbsp;Valeria Buccheri ,&nbsp;Mihoko Yamamoto ,&nbsp;Sergio Costa Fortier ,&nbsp;Maura Rosane Valerio Ikoma-Colturato ,&nbsp;Nelson Hamerschlak ,&nbsp;Vera Lucia de Piratininga Figueiredo ,&nbsp;Talita Maira Bueno da Silveira ,&nbsp;Abel Costa ,&nbsp;Dani Laks ,&nbsp;Rony Schaffel ,&nbsp;Wolney Gois Barreto ,&nbsp;Adriana Scheliga ,&nbsp;Pedro Amoedo Fernandes ,&nbsp;Samir Kanaan Nabhan ,&nbsp;Rafael Dezen Gaiolla ,&nbsp;Matheus Vescovi Gonçalves ,&nbsp;Danielle Leão Cordeiro de Farias ,&nbsp;Glaciano Ribeiro ,&nbsp;Celso Arrais-Rodrigues","doi":"10.1016/j.htct.2025.103822","DOIUrl":"10.1016/j.htct.2025.103822","url":null,"abstract":"<div><div>Chronic lymphocytic leukemia, characterized by an accumulation of monoclonal B lymphocytes, is the most common adult leukemia. The disease predominantly affects older adults, with a significant proportion being asymptomatic at diagnosis. This manuscript provides a comprehensive review of chronic lymphocytic leukemia, including its epidemiology, clinical presentation, diagnostic criteria, and treatment strategies. Prognostic factors, particularly <em>IGHV</em> mutation status and chromosomal abnormalities, are discussed as critical determinants of disease behavior and treatment response. Recent advances in targeted therapies, such as Bruton's tyrosine kinase inhibitors (BTKi) and B-cell lymphoma 2 inhibitors (BCL-2i), have changed the treatment landscape by demonstrating superior efficacy to chemoimmunotherapy. However, disparities in access to care, particularly in low- and middle-income countries such as Brazil, highlight the need for equitable treatment approaches. The discussion of measurable residual disease (MRD) assessment for prognostication and treatment planning is also highlighted. This review highlights the need for continued research and integration of novel therapies to optimize patient outcomes in chronic lymphocytic leukemia.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103822"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143924838","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-HLA antibody formation increases the chances of platelet refractoriness in platelet-transfused patients: a systematic review with meta-analysis","authors":"Luana Joana Barreto Cabral ,&nbsp;Daniela Pereira Lopes ,&nbsp;Eduardo dos Santos Martins Filho ,&nbsp;Rubenilson Caldas Valois ,&nbsp;Paula Christine Amarantes Justino Oliveira ,&nbsp;Patrícia Jeanne de Souza Mendonça-Mattos","doi":"10.1016/j.htct.2025.103821","DOIUrl":"10.1016/j.htct.2025.103821","url":null,"abstract":"<div><div>Platelet refractoriness caused by alloimmunization to anti-HLA antibodies remains present in daily hemotherapy: the frequent need for platelet transfusions may influence the long-term survival of treated patients. This study aimed to perform a systematic review with meta-analysis to investigate the chances of anti-HLA antibody formation triggering immune-induced platelet refractoriness in platelet transfused individuals. By adopting Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria, a search was conducted of publications in online databases between 1976 and July 2022. The risk of bias in the studies was assessed according to the data quality assessment proposed by the ‘A MeaSurement Tool to Assess systematic Reviews’ (AMSTAR-2) tool. Meta-analysis was performed by evaluating the Forest and Funnel Plots. From 832 published articles, 50 were read in full with 14 studies being included in this systematic review. The forest plot showed a likely low heterogeneity (I²: 12.3%; p-value = 0.32), and high odds ratio (174.57; confidence interval: 73.23–416.16) showing platelet refractoriness is triggered by anti-HLA alloantibodies. In this study, anti-HLA antibody formation contributed to an approximate 175-fold higher chance of triggering immune-induced platelet refractoriness. Some explanations about why some statistical differences were observed are offered by studies. This study demonstrates the need for developing policies to identify and monitor anti-HLA antibodies in patients, as well as for HLA matching, and makes some suggestions for future research to promote the prevention of patient sensitization due to platelet transfusions including the development of platelet refractoriness.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103821"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143833684","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hybrid histone deacetylase-kinase inhibitor potentiates venetoclax-induced cell death in chronic lymphocytic leukemia","authors":"Anali Del Milagro Bernabe Garnique ,&nbsp;Jorge Antonio Elias Godoy Carlos ,&nbsp;Natalia Sudan Parducci ,&nbsp;Mauricio Temotheo Tavares ,&nbsp;Karoline de Barros Waitman ,&nbsp;Keli Lima ,&nbsp;Leticia Veras Costa-Lotufo ,&nbsp;Roberto Parise-Filho ,&nbsp;João Agostinho Machado-Neto","doi":"10.1016/j.htct.2025.103757","DOIUrl":"10.1016/j.htct.2025.103757","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103757"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143747287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Problems with single platforms for CD34+ quantification: How aware are Brazilian hematologists and transplant specialists about them?","authors":"Daniel Mazza Matos","doi":"10.1016/j.htct.2025.103836","DOIUrl":"10.1016/j.htct.2025.103836","url":null,"abstract":"","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103836"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143874080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing the genetic profile of cytochrome P450 and glutathione S-transferases of patients diagnosed with acute myeloid leukemia","authors":"Gilmar de Andrade França ,&nbsp;Luciana Nardinelli ,&nbsp;Ricardo Rodrigues Giorgi ,&nbsp;Thiago Pagliarini ,&nbsp;Otávio César Carvalho Guimarães Baiocchi ,&nbsp;Elvira Deolinda Rodrigues Pereira Velloso ,&nbsp;Wellington Fernandes da Silva Jr ,&nbsp;Eduardo Magalhães Rego ,&nbsp;Israel Bendit","doi":"10.1016/j.htct.2025.103759","DOIUrl":"10.1016/j.htct.2025.103759","url":null,"abstract":"<div><h3>Objective</h3><div>This study aimed to determine the frequency of genetic alterations as deletions and duplications in cytochrome P450 (<em>CYP450</em>) and glutathione S-transferases (<em>GST</em>) genes, as well as to investigate whether there is a relationship between these alterations and neutrophilic hematologic recovery in adult patients diagnosed with acute myeloid leukemia.</div></div><div><h3>Method</h3><div>DNA samples from 70 patients diagnosed with acute myeloid leukemia were evaluated using the Multiplex Ligation-dependent Probe Amplification technique. The presence or absence of polymorphisms was compared regarding the time to neutrophilic recovery (neutrophil count ≥1.0 × 10⁹/L) using Kaplan-Meier curves, with the comparison between the curves being performed using the non-parametric log-rank test.</div></div><div><h3>Results</h3><div>The median age of the participants was 57 years, with a higher proportion of females (57.2%) and white individuals (61.4%)’. A total of 76 polymorphisms (<em>CYP450</em> + <em>GST</em>) were identified, comprising 38 deletions and 38 duplications. Kaplan-Meier curves revealed that the neutrophilic recovery time was longer for the group with polymorphisms (p-value = 0.0056).</div></div><div><h3>Conclusion</h3><div>The study demonstrated that <em>CYP450</em> and <em>GST</em> genes are polymorphic, and these polymorphisms may lead to longer neutrophilic recovery after induction treatment of acute myeloid leukemia remission.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103759"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143912632","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and laboratorial characterization of a cohort of patients with hereditary platelet disorders in Brazil","authors":"Letícia Dalla Vecchia Grassi ,&nbsp;Erica Okazaki ,&nbsp;Cynthia Rothschild ,&nbsp;Paula Villaça ,&nbsp;Fernanda Andrade Orsi ,&nbsp;Bianca Stefanello","doi":"10.1016/j.htct.2025.103837","DOIUrl":"10.1016/j.htct.2025.103837","url":null,"abstract":"<div><h3>Introduction</h3><div>Inherited platelet disorders are rare conditions characterized by altered platelet function and/or reduced platelet counts. Diagnosing these disorders is challenging and may result in delays, misdiagnosis, and inappropriate treatment. In low- and middle-income countries, data are scarce. Here, we describe a cohort of patients at a reference center in Brazil.</div></div><div><h3>Methods</h3><div>A descriptive analysis was conducted on patients followed at the Thrombosis and Hemostasis outpatient clinic of the Hospital das Clinicas, University of São Paulo, Brazil.Medical records of 857 patients with thrombocytopenia or bleeding disorders of unknown cause, evaluated between 1998 and 2023, were reviewed. Of these, 60 patients had a confirmed or suspected diagnosis of an inherited platelet disorder and were included in the study.</div></div><div><h3>Results</h3><div>Among the 60 patients, the majority were female (75 %), with a median age of 48 years. The suspicion of a platelet disorder was based on clinical presentation, family history, and laboratory findings. Overall, 65 % of the patients had abnormal platelet function, while 35 % presented with thrombocytopenia. A positive family history was reported in 62 % of those with low platelet counts and in 51 % of patients with platelet function abnormalities. Previous misdiagnoses included immune thrombocytopenia and von Willebrand disease. Overall, the bleeding phenotype was mild, with a median ISTH-BAT (International Society on Thrombosis and Haemostasis Bleeding Assessment Tool) score of 6. Patients with reduced platelet counts tended to have lower ISTH-BAT score.</div></div><div><h3>Conclusions</h3><div>Identifying inherited platelet disorders is essential for proper treatment and follow-up. This study emphasizes the need for careful assessment of family history, bleeding risk, platelet count, morphology, and function for diagnosis, particularly in low-resource settings without access to advanced genetic testing.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103837"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143882266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of fibrin on the expression of adhesion molecules (ICAM-1, ITGAV, and ITGB3) in unrestricted somatic stem cells","authors":"Sanaz Khaseb ,&nbsp;Mahdi Kohansal Vajari ,&nbsp;Mina Soufi Zomorrod ,&nbsp;Maryam Rezai Rad ,&nbsp;Monireh Ajami ,&nbsp;Mansoureh Ajami ,&nbsp;Saba Sadeghpour ,&nbsp;Amir Atashi","doi":"10.1016/j.htct.2025.103827","DOIUrl":"10.1016/j.htct.2025.103827","url":null,"abstract":"<div><h3>Background</h3><div>Hematopoietic stem cell expansion relies on direct cell-cell interactions mediated by adhesion molecules, integrins, and cytokines. Unrestricted somatic stem cells have emerged as novel stromal cells supporting hematopoietic stem cell expansion in co-culture conditions via secretion of hematopoiesis-related cytokines and the expression of adhesion molecules. Previous research showed fibrin increased hematopoiesis-related gene expression in these cells. This study focused on the adhesive characteristics of unrestricted somatic stem cells on 3D fibrin scaffolds.</div></div><div><h3>Methods</h3><div>Unrestricted somatic stem cells were isolated from umbilical cord blood and characterized using flow cytometry and multilineage differentiation assays. Scanning electron microscopy and DAPI staining were employed to analyze cell attachment to fibrin. Viability on fibrin was assessed through MTT assays. Quantitative polymerase chain reaction was conducted to evaluate the expression of intercellular adhesion molecule 1 (ICAM-1), integrin subunit αv (ITGAV), and integrin subunit β3 (ITGB3) in cells cultured on 3D fibrin scaffolds.</div></div><div><h3>Results</h3><div>Cells were positive for CD73, CD105, and CD166 but negative for CD45. Alizarin red and Oil red O stains confirmed calcium deposition and lipid vacuoles. MTT assays revealed that fibrin positively impacts viability. ITGAV expression was significantly increased in cells cultured on fibrin compared to those cultured on plastic tissue culture plates (Control Group). Furthermore, ITGB3 expression showed no significant change in both groups, while ICAM-1 expression was downregulated in cells cultured on fibrin.</div></div><div><h3>Conclusions</h3><div>Our study revealed that fibrin has a positive impact on the expression of ITGAV, which plays a crucial role in direct cell-cell interactions affecting hematopoietic stem cell expansion.</div></div>","PeriodicalId":12958,"journal":{"name":"Hematology, Transfusion and Cell Therapy","volume":"47 2","pages":"Article 103827"},"PeriodicalIF":1.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143895027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}