Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 15 288 294 EN Pouya Vakilipour Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Ayda Firouzabadi Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Student Research Committee, Afzalipour Faculty of Medicine, Kerman University of Medical Sciences, Kerman, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 03 12 2025 03 15 Background: Ataxia-telangiectasia (AT) is a rare, autosomal recessive neurodegenerative disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, recurrent infections, radiosensitivity, and an increased risk of malignancies. This study aimed to evaluate the distribution of A-T patients, parenteral consanguinity status, and diagnostic delays in different provinces of Iran. Methods: A retrospective observational and analytical study in which all A-T patients with a recorded place of birth or residency were included. A questionnaire was designed and filled out for each patient to extract data including date of birth, gender, parental consanguinity status, family history, age of disease onset, and age of disease diagnosis. Results: A total of 203 A-T patients (104 males and 99 females) were included in the study. Out of a total of 31 provinces, A-T patients were diagnosed and reported in 25 different provinces, while six provinces had no registered A-T patients. Tehran province, the most densely populated province in Iran, reported the predominant number and frequency of cases (52 patients or 25.6%), followed by Khouzestan (16 cases, 7.9%), Alborz (12 cases, 5.9%) and Isfahan (12 cases, 5.9%) provinces. No statistically significant relationship was found regarding family history status. Hamedan followed by Sistan and Baluchestan, and Yazd provinces had the highest delay in diagnosis. Conclusion: Our study showed that A-T is distributed in most provinces of Iran. We found a considerably high diagnostic delay among A-T patients in Iran, especially in resource-limited provinces, including Hamedan followed by Sistan and Baluchestan, and Yazd. https://igj.tums.ac.ir/index.php/igj/article/view/205 https://igj.tums.ac.ir/index.php/igj/article/download/205/188

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 15 258 266 Samaneh Delavari Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Hassan Abolhassani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Fereshte Salami Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran Samaneh Abdolahzadeh Department of Pediatric Diseases, School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran Parisa Ashournia Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Aida Askarisarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; Assistant Professor, Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Mohammad Hassan Bemanian Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran Kian Darabi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran Sarehsadat Ebrahimi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Shabnam Eskandarzadeh Department of pediatrics, Pediatric Health Center, Tabriz University of Medical Sciences, Tabriz, Iran Golnaz Eslamian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Narges Eslami Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahrzad Fallah Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Morteza Fallahpour Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Saba Fekrvand Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Ali Haghbin Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran Zohre Hassanpoor Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Tolue Mahdavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center Hospital, Tehran, Iran Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mohammamd Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mojtaba Ranjbar Pediatric Center of Excellence, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Anahita Razaghian Division of Allergy and Clinical Immunology, Department of Pediatrics, Hakim Children Hospital, Tehran University of Medical Sciences, Tehran, Iran؛ Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. Mohammad Saberi Department of Medical Genetics, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Mahnaz Sadeghi-Shabestari Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran Mitra Sahragard Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Kiarash Saleki Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Sahar Seraj Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran Seyedehshabnam Seyedsalehi Abuzar Children’s Medical Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Alireza Shafiei Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran Roya Sherkat Acquired Immunodeficiency Research Center, lsfahan University of Medical Sciences, Isfahan, lran Reza Shiari Division of Pediatric Rheumatology, Department of Pediatrics, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Saman Tavakoli Department of Pediatric, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Ahmad Vosughimotlagh Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Niloufar Yazdanpanah Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 03 12 2025 03 15 Currently, a national consensus or guideline for diagnosing and managing patients suspected of having chronic granulomatous disease (CGD) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunologists reviewed the current consensus, presented their comments at a meeting titled “First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts” and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, administration of interferon gamma (IFN-γ) and hematopoietic stem cell transplantation (HSCT) for patients with CGD. https://igj.tums.ac.ir/index.php/igj/article/view/207 https://igj.tums.ac.ir/index.php/igj/article/download/207/185

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 12 303 307 Shayan Roshdi School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Medical Genetics Network (MeGeNe), Universal Scientific Education and Research Network (USERN), Munich, Germany Sepideh Shahkarami Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-Universität München, Munich, Germany; Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Samaneh Zoghi Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; St. Anna Children's Cancer Research Institute (CCRI), Vienna, Austria Elham Rayzan Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; 4 Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran Rasol Molatefi Pediatric department of Bou-Ali Educational Hospital, Ardabil University of Medical Sciences, Ardabil, Iran Meino Rohlfs Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-Universität München, Munich, Germany Christoph Klein Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-Universität München, Munich, Germany Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 01 16 2025 03 12 WHIM syndrome (Warts, Hypogammaglobulinemia, Immunodeficiency, and Myelokathexis syndrome), a type of severe congenital neutropenia (SCN), involves Warts, Hypogammaglobulinemia, Infections, and Myelokathexis as its main components of clinical presentation, which results from mutations in the C-X-C chemokine receptor type 4 (CXCR4) gene. Here, we present an Iranian 4-year-old girl with severe congenital neutropenia without warts and normal bone marrow examination, lacking evidence of myelokathexis. Whole Exome Sequencing (WES) was performed for the patient. Subsequently, Sanger segregation/validation was done of the patient and her parents. Whole exam sequencing identified a heterozygous stop variant mutation in CXCR4 (NM_001008540.2:c.1012C>T; p.Arg338Ter) in the patient. Two of the main clinical criteria in WHIM syndrome, including warts and myelokathexis, were not observed in our patient. So, the absence of warts is not deceptive in ruling out the disease. This case report also represents the high importance of genetic analysis as a primary tool for the accurate differential diagnosis of patients with neutropenia. https://igj.tums.ac.ir/index.php/igj/article/view/168 https://igj.tums.ac.ir/index.php/igj/article/download/168/190

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 15 256 257 Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 03 12 2025 03 15 No Abstract https://igj.tums.ac.ir/index.php/igj/article/view/204 https://igj.tums.ac.ir/index.php/igj/article/download/204/183

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 15 295 302 Mahsima Shabani Research Center for Immunodeficiencies, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; International Hematology/Oncology of Pediatrics Experts (IHOPE), Universal Scientific Education and Research Network (USERN), USA Elham Rayzan Research Center for Immunodeficiencies, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; International Hematology/Oncology of Pediatrics Experts (IHOPE), Universal Scientific Education and Research Network (USERN), USA Ido Somekh Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig Maximilians University, Munich, Germany Christoph Klein Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig Maximilians University, Munich, Germany Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 03 12 2025 03 15 Background: Congenital cardiac anomalies are considered the most frequent non-hematologic manifestation of Glucose-6-phosphatase 3 (G6PC3) deficiency. Methods: We report a case of G6PC3-deficiency with a novel homozygous frameshift variant (c.911dupC; p.Gln305SerfsTer82), who developed intermittent neutropenia and was diagnosed long after a repair cardiac surgery for patent ductus arteriosus (PDA). To further investigate the importance of immunologic workups in patients with congenital cardiac defects, we provide a literature review on the observed cardiac findings in patients with SCN4. Results: Overall, 78.3% of reported patients had cardiac defects, with more than half of the patients (56%) presenting with ASD. More than half of the patients with ASD required surgical repair, which implies the severity of symptoms. Conclusion: These findings highlight the importance of performing immunologic work-ups in children initially manifesting congenital heart defects. A simple differential cell-blood-count test may prevent future life-threatening disseminated infections, especially in countries with high rates of consanguinity and, subsequently, higher prevalence of primary immunodeficiencies. https://igj.tums.ac.ir/index.php/igj/article/view/206 https://igj.tums.ac.ir/index.php/igj/article/download/206/189

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 15 267 272 Saba Fekrvand Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Samaneh Abdolahzade Department of Pediatric disease, School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran Parisa Ashournia Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Aida Askarisarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; Assistant Professor, Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Zahra Daneshmandi Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran Sarehsadat Ebrahimi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Shabnam Eskandarzadeh Department of pediatrics, Pediatric Health Center, Tabriz University of Medical Sciences, Tabriz, Iran Narges Eslami Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Golnaz Eslamian Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahrzad Fallah Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Ali Haghbin Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mohammamd Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Anahita Razaghian Division of Allergy and Clinical Immunology, Department of Pediatrics, Hakim Children Hospital, Tehran University of Medical Sciences, Tehran, Iran؛ Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran. Mahnaz Sadeghi-Shabestari Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran Sahar Seraj Department of Immunology and Allergy, Shahid Beheshti University of Medical Sciences, Tehran, Iran Seyedehshabnam Seyedsalehi Abuzar Children’s Medical Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Alireza Shafiei Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran Reza Shiari Division of Pediatric Rheumatology Department of Pediatrics Mofid Children's Hospital Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Saman Tavakoli Department of Pediatric, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Ahmad Vosughimotlagh Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Vahid Ziaee Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 03 15 2025 03 15 At present, a national consensus or guideline for diagnosing and managing patients suspected of having severe congenital neutropenia (SCN) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunologists reviewed the current consensus, presented their comments at a meeting titled “First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts” and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, administration of granulocyte colony-stimulating factor (G-CSF) and hematopoietic stem cell transplantation (HSCT) for patients with SCN. https://igj.tums.ac.ir/index.php/igj/article/view/208 https://igj.tums.ac.ir/index.php/igj/article/download/208/186

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 8 3 2025 03 12 273 287 Fatemeh Tajik Oncopathology Research Center, Iran University of Medical Sciences, Tehran, Iran; Cancer Immunology Project (CIP), Universal Scientific Education and Research Network (USERN), Tehran, Iran Sara Kamali Zonouzi Tehran University of Medical Sciences Sepideh Razi Cancer Immunology Project (CIP), Universal Scientific Education and Research Network (USERN), Tehran, Iran; Research Center for Immunodeficiencies, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran 2025 03 02 2025 03 12 Pancreatic cancer is still one of the most lethal malignancies across the world, and hence exploring new biomarkers related to the progression and invasive nature of this cancer is important to overcome its resistance to various types of treatments through the design of new therapeutic strategies. Several markers have been shown to play a role in pancreatic cancer invasion, but CA19-9, CA125, and noncoding RNAs, including microRNAs, long noncoding RNAs, and circular RNAs, are the most common ones. In the current review, the role of these markers in pancreatic cancer progression, invasion, and metastasis, as well as related mechanisms, has been provided, and their potential to be utilized in pancreatic cancer diagnosis and treatment has been discussed. https://igj.tums.ac.ir/index.php/igj/article/view/201 https://igj.tums.ac.ir/index.php/igj/article/download/201/187