Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 24 6 2025 10 29 799 807 EN Maryam Moradi Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Mahsa Yousefpour Marzbali Research Center for Immunodeficiencies, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Nazanin Nazari Shiraz Institute for Cancer Research, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran Zahra Chavoshzadeh Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Seyed Alireza Mahdaviani St. Giles Laboratory of Human Genetics of Infectious Disease, The Rockefeller University, New York, NY, USA AND Paris Cite University, Imagine Institute, Paris, France, EU AND Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahnaz Sadeghi-Shabestari Immunology Research Center of Tabriz, Mardaniazar Children Hospital of Tabriz, Tabriz University of Medical Sciences, Tabriz, Iran Nima Rezaei Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran AND Research Center for Immunodeficiencies, Children’s Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran AND Network for Immunology in Infection, Malignancy, And Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2024 12 04 2025 04 23   The autosomal recessive form of hyperimmunoglobulin E syndrome (AR-HIES), caused by mutations in the DOCK8 (Dedicator of Cytokinesis 8) gene, presents a wide range of clinical manifestations and phenotypically overlaps with several types of combined immunodeficiency disorders characterized by elevated serum IgE levels. Due to the high rates of morbidity and mortality, as well as the potential curability through hematopoietic stem cell transplantation (HSCT), early and accurate differential diagnosis of this syndrome is crucial for optimal management and improved prognosis. Flow cytometry tests can be beneficial for early diagnosis of many inborn errors of immunity (IEIs), including this syndrome. This study, conducted for the first time on Iranian patients, investigated the expression of the DOCK8 protein. DOCK8 expression was assessed by flow cytometry in 14 patients (6 males and 8 females) with a clinical diagnosis of DOCK8 deficiency. The diagnosis was ultimately confirmed through genetic testing. The results showed that DOCK8 expression in patients was significantly lower compared to the healthy control group. Flow cytometric evaluation of DOCK8 protein expression offers a rapid and efficient diagnostic method with a sensitive detection range suitable for many cases. This approach can facilitate the diagnosis of DOCK8 deficiency, thereby enabling timely and effective disease management.  https://ijaai.tums.ac.ir/index.php/ijaai/article/view/4267 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/4267/2246