Padideh Oghab, Ali Erfan, Mohadese Poorpoone, Mohammad Saleh Peikar
{"title":"Feasibility and Effectiveness of CALGB 10403 Protocol in Adolescent and Young Adult Patients with Acute Lymphoblastic Leukemia/ Lymphoblastic Lymphoma: A Study at Seyed Al-Shohada Hospital, Isfahan, Iran.","authors":"Padideh Oghab, Ali Erfan, Mohadese Poorpoone, Mohammad Saleh Peikar","doi":"10.18502/ijhoscr.v20i1.21010","DOIUrl":"10.18502/ijhoscr.v20i1.21010","url":null,"abstract":"<p><p><b>Background:</b> Acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) are rare blood cancers with poorer survival in adolescents and young adults (AYAs) than in children. Pediatric-inspired regimens like CALGB 10403 have improved outcomes in AYAs. This study evaluated the effectiveness, feasibility, and treatment-related toxicities of CALGB 10403 in AYAs with ALL/LBL treated at Seyed Al-Shohada Hospital, Isfahan, Iran, from December 2021 to May 2024 <b>Materials and Methods:</b> AYAs aged 17-39 with newly diagnosed ALL/LBL (excluding Burkitt, Ph+, and prior treatment) were included. Baseline characteristics and outcomes of interest (induction response, event-free and overall survivals) were collected. Induction response was assessed via bone marrow (ALL) or CT scan/biopsy (LBL). Minimal residual disease (MRD) was evaluated by flow cytometry. Treatment-related toxicities were monitored and graded per CTCAE criteria. The feasibility of implementing the CALGB 10403 protocol was evaluated by measuring treatment delays. <b>Results:</b> Seventeen AYA patients with newly diagnosed ALL/LBL (median age 21) were enrolled, mostly male (14/17). Fourteen had ALL, and three had LBL. Extramedullary involvement was seen in 29%, including lymphadenopathy, bulky mediastinal masses, and CNS involvement. Sixteen patients (94%) achieved remission, with no deaths during the induction course. One-year EFS and OS were 77% and 100%, respectively. Common toxicities included hyperbilirubinemia, elevated transaminases, and infections. Treatment delays, mainly from non-adherence, occurred in 53%. Eight patients achieved MRD negativity after Course I. <b>Conclusion:</b> This study provides insights into the first Iranian experience with the CALGB 10403 protocol for AYAs with ALL/LBL, a regimen that has demonstrated encouraging survival outcomes in U.S. trials.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"20 1","pages":"14-29"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13249071/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148221416","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}
Mahdi Alaee, Meysam Moulaee, Kimia Taebi, Ahoura Haghi, Maryam Hormozi, Mehdi Azad
{"title":"The Role of miRNA-21 in the Metastasis of Hepatocellular Carcinoma as a Therapeutic Target.","authors":"Mahdi Alaee, Meysam Moulaee, Kimia Taebi, Ahoura Haghi, Maryam Hormozi, Mehdi Azad","doi":"10.18502/ijhoscr.v20i1.21013","DOIUrl":"10.18502/ijhoscr.v20i1.21013","url":null,"abstract":"<p><p>Hepatocellular carcinoma (HCC) is a crucial health concern worldwide, representing a leading cause of cancer-related mortality and the most common form of primary liver cancer. The aggressive nature of HCC is mainly due to its high tendency for invasion and metastasis, processes regulated by a complex network of genetic and molecular pathways. Among the critical regulators of these processes is microRNA-21 (miR-21), a small non-coding RNA that implicated in various oncogenic activities. This review provides a comprehensive analysis of the role of miR-21 in promoting HCC metastasis, with a particular focus on its interaction with key signaling pathways, including the PTEN/PI3K/AKT, PDCD4/AP-1, RECK/MMP, and TIMP-3 axes. By targeting tumor suppressors, miR-21 facilitates epithelial-to-mesenchymal transition (EMT), invasion, and metastasis of HCC cells. Understanding the molecular mechanisms regulated by miR-21 not only sheds light on the pathogenesis of HCC but also highlights potential therapeutic targets for combating this aggressive cancer.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"20 1","pages":"46-58"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13255715/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148255064","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}
Mohammad Jahangiri, Suzanne Hojjat-Assari, Pooneh Pirjani, Mohammad Vaezi, Ghasem Janbabaei, Shayesteh Kokabi Hamidpour, Nafiseh Heidarzadeh Isfahani, Babak Arjmand
{"title":"Establishment of Integrated Hospital-Based Supportive and Palliative Services: An Experience from Iran.","authors":"Mohammad Jahangiri, Suzanne Hojjat-Assari, Pooneh Pirjani, Mohammad Vaezi, Ghasem Janbabaei, Shayesteh Kokabi Hamidpour, Nafiseh Heidarzadeh Isfahani, Babak Arjmand","doi":"10.18502/ijhoscr.v20i1.21015","DOIUrl":"10.18502/ijhoscr.v20i1.21015","url":null,"abstract":"<p><p>Integrating supportive and palliative care into the oncology program is considered essential for effective cancer management. Despite the challenges that low- and middle-income countries such as Iran face in providing comprehensive supportive and palliative care services, continuous efforts are directed towards improving services and infrastructure. In this regard, the Iranian Cancer Control Center (MACSA) plays a fundamental role in providing supportive and palliative care with special emphasis on expanding services and ensuring equitable access for cancer patients in Iran. Through joint efforts with various organizations and hospitals, coupled with the implementation of effective leadership strategies, MACSA has tried to integrate its services into a referral hospital system in order to continuously improve and expand services and meet the multifaceted needs of patients and families affected by cancer. Indeed, MACSA's initiatives to embed supportive and palliative care within the framework of usual care in referral hospitals are critical in improving outcomes and quality of life for people battling cancer. The interdisciplinary nature of supportive and palliative care, involving a specialized team, is essential for efficient service delivery, cost-effectiveness, and overall quality of care.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"20 1","pages":"78-96"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13249029/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148221419","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":"Hybrid Ganciclovir/Valacyclovir Prophylaxis Reduces CMV Reactivation in High-Risk Allogeneic Stem Cell Transplant Recipients.","authors":"Maryam Barkhordar, Amirabbas Rashidi, Mohammad Vaezi, Neda Alijani, Seied Asadollah Mousavi, Sahar Tavakoli Shiraji, Mehrdad Abbaszadeh, Hosein Kamranzadeh Fumani, Tanaz Bahri","doi":"10.18502/ijhoscr.v19i4.19979","DOIUrl":"10.18502/ijhoscr.v19i4.19979","url":null,"abstract":"<p><p><b>Background:</b> Cytomegalovirus (CMV) reactivation remains a critical concern following allogeneic hematopoietic stem cell transplantation (allo-HSCT), particularly in CMV-seropositive patients undergoing allo-HSCT from alternative donors. This study explored whether a hybrid CMV prophylaxis regimen would be more effective than the standard preemptive regimen in resource-limited settings where Letermovir is unavailable or cost-prohibitive. <b>Materials and Methods:</b> This prospective single-center cohort study included adult patients with acute leukemia who received allo-HSCT from alternative donors between November 2018 and May 2022. The primary outcome was the evaluation of the CMV reactivation incidence in allo-HSCT patients receiving the hybrid CMV prophylaxis regimen comprising pretransplant ganciclovir followed by high-dose valacyclovir compared with the control patients who received the preemptive regimen. Secondary outcomes included overall survival (OS), disease-free survival (DFS), GVHD-free relapse-free survival (GRFS), and non-relapse mortality (NRM) between the two groups. <b>Results:</b> A total of 80 patients, 34 receiving hybrid CMV prophylaxis and 46 receiving the preemptive protocol. The hybrid prophylaxis group exhibited a significantly lower incidence of CMV reactivation at 90 days post-transplantation (34% vs. 82%, <i>P</i> = 0.000). However, no statistically significant differences were observed in the overall survival, disease-free survival, or non-relapse mortality. <b>Conclusion:</b> The hybrid regimen reduced CMV reactivation in high-risk HSCT recipients but did not improve survival outcomes, offering a practical alternative in settings with limited access to Letermovir.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"310-319"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135746/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847866","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":"Safety and Efficacy of Direct Oral Anticoagulants Compared to Warfarin in Patients with Venous Thromboembolism and Morbid Obesity.","authors":"Anjan Katel, Sujan Niraula, Madan Aryal, Niraj Neupane, Arun Neupane, Vijaya Raj Bhatt","doi":"10.18502/ijhoscr.v19i4.19980","DOIUrl":"10.18502/ijhoscr.v19i4.19980","url":null,"abstract":"<p><p><b>Introduction:</b> Several guidelines recommend using direct oral anticoagulants (DOACs) over warfarin for the prevention of venous thromboembolism (VTE), except in cases of morbid obesity. The use of DAOCs in patients with morbid obesity has not been thoroughly evaluated due to a lack of representation in large clinical trials. To address this knowledge gap, we conducted a systematic review and meta-analysis of existing literature to determine the efficacy and safety of DOACs in patients with morbid obesity. <b>Materials and Methods:</b> A systematic review of studies comparing DOACs with warfarin in patients with morbid obesity and diagnosed with acute VTE was conducted using electronic literature searches up to December 2021. Efficacy and safety were defined as the rate of recurrent VTE and major bleeding, respectively. <b>Results:</b> A total of 30 822 patients were included across 13 studies. Recurrent VTE events were observed in 713 of 12 945 patients treated with DOACs, compared to 966 of 17 877 patients treated with warfarin (OR, 0.70; 95% CI, 0.50-0.99; <i>P</i>=0.04; <i>I</i> <sup>2</sup>=69%). Major bleeding events occurred in 195 of 12 675 patients on DOACs and in 386 of 17 572 patients on warfarin (OR, 0.69; 95% CI, 0.58-0.82; <i>P</i><0.0001; <i>I</i> <sup>2</sup>=0%). Similar findings were noted for two specific DOACS, apixaban and rivaroxaban, when evaluated individually against warfarin. <b>Conclusion:</b> Our meta-analysis indicated that DOACs are both safe and effective in preventing VTE in patients with morbid obesity. DAOCs resulted in comparable outcomes to those seen in warfarin use. This analysis consolidates extensive observational data from a large patient cohort, thereby enhancing the evidence base for the use of DOACs in patients with morbid obesity.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"320-331"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135743/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847937","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":"Comparative Diagnostic Performance of Flow Cytometry, Aspiration, and Biopsy with Immunohistochemistry in Plasma Cell Neoplasms.","authors":"Alireza Moazedi, Samaneh Salarvand, Zahra Salehi, Alireza Zangooie, Leyla Sahebi, Alireza Abdollahi, Fatemeh Nili, Fereshteh Ameli","doi":"10.18502/ijhoscr.v19i4.19984","DOIUrl":"10.18502/ijhoscr.v19i4.19984","url":null,"abstract":"<p><p><b>Background:</b> Plasma cell neoplasms (PCNs) are a heterogeneous group of hematologic malignancies that require accurate and timely diagnosis for effective management. Despite the availability of multiple diagnostic tools, challenges remain due to clinical and morphological variability. This study aimed to compare the diagnostic performance of three key modalities, including flow cytometry (FCM), bone marrow aspiration (BMA), and bone marrow biopsy with immunohistochemistry (BMB+IHC), in patients with plasma cell neoplasms. <b>Materials and Methods:</b> A cross-sectional study was conducted on 52 patients with confirmed PCNs. Diagnostic outcomes from FCM, BMA, and BMB+IHC were evaluated and compared. Sensitivity, specificity, predictive values, and inter-method agreement were calculated using SPSS version 27. <b>Results:</b> BMB+IHC achieved the highest diagnostic yield (100%), followed by BMA (55.8%), while FCM demonstrated the lowest diagnostic rate (32.7%). Flow cytometry showed excellent specificity and a positive predictive value of 100%, but limited sensitivity (32.7-58.6%), resulting in a high rate of false negatives. BMA frequently underestimated plasma cell burden due to sampling variability and hemodilution. Collectively, the integration of all three methods provided complementary diagnostic value, reducing the risk of misclassification. <b>Conclusion:</b> Bone marrow biopsy with immunohistochemistry remains the gold standard for diagnosing PCNs. However, combining it with aspiration and flow cytometry offers a more comprehensive diagnostic framework, improving accuracy, minimizing false negatives, and supporting optimal patient management.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"354-364"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135741/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847824","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":"Genetic Engineering in Hematopoietic Stem Cells for β-Hemoglobinopathies Treatment: Advances, Challenges, and Clinical Translation.","authors":"Sahar Taebi, Fatemeh Eskandari, Masoumeh Kohandani, Tahereh Manoochehrabadi, Hajar Nasiri","doi":"10.18502/ijhoscr.v19i4.19988","DOIUrl":"10.18502/ijhoscr.v19i4.19988","url":null,"abstract":"<p><p>β-hemoglobinopathies rank among the most prevalent inherited blood disorders globally. Traditional management strategies are primarily palliative and often associated with significant challenges, including iron overload and limited long-term efficacy. Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative option for transfusion-dependent patients, but its broader applicability is constrained by factors that limit its use. Utilizing viral vectors and gene-editing tools, particularly CRISPR-Cas9 technology, researchers have developed therapies that target the root causes of these disorders. These innovative approaches have demonstrated substantial therapeutic potential, accompanied by favorable safety profiles, in clinical settings. Since the initial investigations, the genome editing tool has rapidly advanced for genetic abnormalities, particularly monogenic blood diseases, including β-hemoglobinopathies. This method suggests an approach with lower concerns in viral gene integration and insertional mutagenesis issues. This review comprehensively surveys the therapeutic strategies for β-thalassemia and sickle cell disease (SCD) currently in preclinical and clinical development, with a focus on the evolving treatment paradigm. Looking forward, critical research priorities include optimizing the efficiency and specificity of gene-editing platforms and pioneering novel delivery systems to guarantee both therapeutic efficacy and clinical safety.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"399-423"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135740/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847893","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}
Osias Vieira de Oliveira-Filho, Yuri de Lima Medeiros, Lorena Dos Santos Girão, Igor Campos Guimarães, Henrique Girão Martins, André Costa Teixeira, Saygo Tomo
{"title":"Ulcerated Tongue Borders as a Sign of Amyloidosis in a Patient with Multiple Myeloma.","authors":"Osias Vieira de Oliveira-Filho, Yuri de Lima Medeiros, Lorena Dos Santos Girão, Igor Campos Guimarães, Henrique Girão Martins, André Costa Teixeira, Saygo Tomo","doi":"10.18502/ijhoscr.v19i4.19990","DOIUrl":"10.18502/ijhoscr.v19i4.19990","url":null,"abstract":"<p><p>Amyloidosis is characterized by extracellular deposition of amyloid material in various tissues and organs. Deposition of amyloid in the tongue is rare but often occurs in multiple myeloma. Here we present the case of a 44-year-old woman under treatment for multiple myeloma who complained of lateral tongue pain. Intraoral examination revealed an ill-defined, firm, whitish swelling with an ulcerated surface on the bilateral borders of the tongue. Histopathological analysis revealed a lympho-mononuclear infiltration in the lamina propria with the presence of acellular and amorphous material positive for Congo red staining. Further examination revealed amyloidosis in a cervical mass associated with neural compression. Thorough examination of the oral cavity in patients with multiple myeloma is critical for early detection of amyloidosis. A biopsy is mandatory to confirm the diagnosis, thereby facilitating intervention and management if necessary.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"429-433"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135734/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847944","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}
Fariba Sarem, Azita Azarkeivan, Ahmad Mardani, Majid Shahabi
{"title":"Expression of the FAM132B Gene in Iranian Patients with Beta-Thalassemia.","authors":"Fariba Sarem, Azita Azarkeivan, Ahmad Mardani, Majid Shahabi","doi":"10.18502/ijhoscr.v19i4.19982","DOIUrl":"10.18502/ijhoscr.v19i4.19982","url":null,"abstract":"<p><p><b>Background:</b> Iron homeostasis is a complex process involving multiple factors. Erythroblasts secrete erythroferrone (ERFE), which affects hepcidin production, thereby enhancing iron uptake. This study aimed to investigate the expression of the ERFE-encoding FAM132B gene in Iranian patients with beta-thalassemia major (BTM) and beta-thalassemia intermedia (BTI). <b>Materials and Methods:</b> A total of 40 BTM and BTI patients and 20 healthy blood donors as a control group were recruited. Total RNA was extracted from whole blood samples, and cDNA was synthesized. Gene expression was quantified using a SYBR Green-based real-time PCR (RT-PCR) assay. Data analysis was performed by the GraphPad Prism program using the one-way ANOVA test. <b>Results:</b> The expression of the FAM132B gene was upregulated in BTI patients compared to BTM patients (<i>p</i> = 0.0151). Despite the higher mRNA fold change in BTI patients, no significant difference was observed in the FAM132B gene expression between beta-thalassemia patients (major and intermedia) and the control group. <b>Conclusion:</b> The expression of the FAM132B gene was different between beta-thalassemia major and intermedia patients. Further studies should be conducted to better elucidate the role of erythroferrone as a potential therapeutic target in patients with beta-thalassemia.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"338-343"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135731/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847791","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}
Ehsan Shahverdi, Corinna Petz, Turadj Hemmati, Alireza Farsinezhad, Ali Heidari, Mohammad Faranoush
{"title":"Apoptotic Events in Type-I Glanzmann Thrombasthenia Platelets.","authors":"Ehsan Shahverdi, Corinna Petz, Turadj Hemmati, Alireza Farsinezhad, Ali Heidari, Mohammad Faranoush","doi":"10.18502/ijhoscr.v19i4.19983","DOIUrl":"10.18502/ijhoscr.v19i4.19983","url":null,"abstract":"<p><p><b>Background</b>: Activated normal platelets undergo numerous biochemical and morphological changes, some of which are apoptotic. Phosphatidylserine (PS) expression, Δψm depolarization, microparticle (MP) formation, platelet shrinkage, release of cytochrome c, and caspase activation are hallmarks of both platelet activation and apoptosis. In this study, we report the apoptotic responses of type-I Glanzmann thrombasthenic platelets. <b>Materials and Methods</b>: Platelets from twelve unrelated patients with type I Glanzmann thrombasthenia were examined as washed platelets. Calcium ionophore A23187 was used as an agonist to activate the platelets. Flow cytometry was employed to detect phosphatidylserine expression (Annexin A5 Alexa Fluor), Δψm depolarization (JC-10), platelet-derived MP formation (forward scatter; events <1.0 µm in size), and platelet shrinkage (mean-FSC). Anti-CD42b was used as a platelet-specific marker to distinguish platelets from other particles. <b>Results</b>: We determined that increased cytosolic calcium significantly increased PS exposure, depolarized mitochondrial inner membrane potential (Δψm), increased microparticle formation, and induced platelet shrinkage in type-I Glanzmann thrombasthenic platelets. Our research showed that type I Glanzmann thrombasthenic platelets exhibit characteristics of platelet apoptosis. GPIIbIIIa deficiency does not limit platelet activation or apoptosis. <b>Conclusion</b>: We conclude that GPIIb-IIIa-independent mechanisms may be involved in the normal apoptosis of thrombasthenic platelets. Our data deepen the understanding of the role of the platelet fibrinogen receptor in revealing aspects of normal apoptosis. However, this may help explain the normal platelet count among thrombasthenic patients.</p>","PeriodicalId":94048,"journal":{"name":"International journal of hematology-oncology and stem cell research","volume":"19 4","pages":"344-353"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13135735/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147847847","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}