Article
Chemistry, Multidisciplinary
Yan Duan, Ling Liang, Fanggui Ye, Shulin Zhao
Summary: In this study, a dual-functional Ce-based metal-organic framework@polydopamine (Ce-MOF@PDA) composite was designed and evaluated for treating iron overload (IO) and oxidative stress injury caused by β-thalassemia. Ce-MOF@PDA showed the ability to scavenge reactive oxygen species (ROS) and eliminate iron overload, reducing tissue damage caused by oxidative stress. The results suggest that Ce-MOF@PDA nanozyme is a promising therapeutic nanomedicine for treating thalassemia IO.
Article
Endocrinology & Metabolism
Arijit Singha, Pradip Mukhopadhyay, Sujoy Ghosh
Summary: This study evaluated the prevalence of adrenal insufficiency (AI) in patients with hemoglobin E/beta thalassemia and its association with disease severity and transfusion requirements. The results showed that AI is common in these patients and is independent of transfusion dependency and disease severity.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2023)
Review
Multidisciplinary Sciences
Mohammad Hassan Hodroj, Ali Taher
Summary: Thalassemia management has advanced with iron chelation therapy, leading to increased life expectancy. However, this has also resulted in the emergence of new morbidities, including cancer. While there is still uncertainty about the mechanisms and risks of cancer development in thalassemia patients, multiple cases have been reported in the literature.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2023)
Article
Hematology
Dinesh Bhurani, Jyotsna Kapoor, Neha Yadav, Vishvdeep Khushoo, Narendra Agrawal, Rayaz Ahmed, Jatinder Singh Arora, Pallavi Mehta
Summary: The retrospective analysis of 140 transplant ineligible TDT patients receiving combination therapy of HU and thalidomide showed that more than half of the patients achieved complete response, with an overall response rate of 71.5%. There was a significant increase in post-treatment hemoglobin and a decrease in serum ferritin among responders.
ANNALS OF HEMATOLOGY
(2021)
Review
Multidisciplinary Sciences
Jeanne Boudreaux
Summary: This article provides an overview of the current challenges and recommendations regarding transfusion management for thalassemia patients. It also discusses the components of blood safety and blood product modification for specific populations. The article describes adverse transfusion reactions and explores newer technologies to minimize the risk of transfusion-associated pathogens. Finally, the article briefly discusses research in blood storage and manufactured blood.
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES
(2023)
Article
Pharmacology & Pharmacy
Xinyu Li, Shuting Hu, Yong Liu, Junjiu Huang, Weicong Hong, Luhong Xu, Honggui Xu, Jianpei Fang
Summary: The study found that a dose of thalidomide between 2.5 mg·kg(-1)·d(-1) and 3.6 mg·kg(-1)·d(-1) is effective in children with TDT, with severe side effects being uncommon. The HbF concentration of 47.298 g·L-1 in the third month of treatment is recommended as the predictor for further major responders.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Public, Environmental & Occupational Health
Anuja P. Premawardhena, Dileepa Senajith Ediriweera, Amir Sabouhanian, Angela Allen, David Rees, Shanthimala de Silva, Windsor Perera, Nimal Katugaha, Mahinda Arambepola, Robert C. Yamashita, Sachith Mettananda, Nilam Jiffry, Vikita Mehta, Refai Cader, Dayananda Bandara, Timothy St Pierre, Giulia Muraca, Christopher Fisher, Abirami Kirubarajan, Shawn Khan, Stephen Allen, Sanath P. Lamabadusuriya, David J. Weatherall, Nancy F. Olivieri
Summary: The study conducted long-term observations on the survival and complications of patients with haemoglobin E thalassaemia, finding that male patients, those with a history of serious infections, and patients with higher estimated body iron burdens had poorer survival rates.
LANCET GLOBAL HEALTH
(2022)
Article
Medicine, General & Internal
Reem Aldwaik, Tamara Abu Mohor, Israa Idyabi, Salam Warasna, Shatha Abdeen, Bashar Karmi, Rania Abu Seir
Summary: Management of beta-thalassemia in the West Bank presents challenges due to the lack of available therapies, leading to recurrent transfusions. Characteristics of patients include anemia, iron overload, and abnormal liver function tests. Iron chelation medication choice is associated with serum ferritin levels, highlighting the need for personalized assessment and follow-up protocols.
FRONTIERS IN MEDICINE
(2021)
Article
Medicine, General & Internal
Funda Eren, Ayca Koca Yozgat, Esra Firat Oguz, Salim Neselioglu, Ridvan Firat, Dilek Gurlek Gokcebay, Huesniye Nese Yarali, Namik Yasar Ozbek, Ozcan Erel
Summary: The aim of this study was to develop new strategies to prevent or reduce organ damage caused by iron-mediated oxidation in thalassemia major patients. Blood samples were taken from 70 patients before and after transfusion and various biomarkers related to oxidation and antioxidant defense were measured. The results showed that transfusion therapy increased both oxidation and antioxidant levels. Additionally, a new parameter called undepleted thiol level was introduced to assess the potential organ damage caused by oxidation in transfusion-dependent thalassemia patients.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Hematology
Nashwa El-Khazragy, Safa Matbouly, Demiana H. Hanna, Nievin Ahmed Mahran, Sally Abdallah Mostafa, Bassam A. Abdelrehim, Yasmeen K. Farouk, Soha Abuelela
Summary: The study identifies an association between three iron-related miRNAs and tissue iron overload in transfusion-dependent beta-thalassemia major patients. Results suggest that circulating miR-let-7d and miR-200b are downregulated, while miR-122 is upregulated in TDT patients. Aberrant expression of miRNAs is significantly associated with increased iron accumulation in hepatic and cardiac tissues, making them potential predictive biomarkers for tissue iron excess.
ANNALS OF HEMATOLOGY
(2021)
Article
Medicine, General & Internal
Sashoy Patterson, Ashley Singleton, Jane Branscomb, Vivien Nsonwu, Regena Spratling
Summary: Chronic transfusion carries risks of complications, but dissemination of evidence-based guidelines is complicated by the small and dispersed thalassemia population. This study assessed thalassemia patients' knowledge of transfusion, their experience of clinicians' following guidelines, and their perspectives on data storage options. The findings highlight the importance of patient education and the implementation of a nationwide registry to improve transfusion safety.
FRONTIERS IN MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Davide Schiroli, Lucia Merolle, Eleonora Quartieri, Roberta Chicchi, Tommaso Fasano, Tiziana De Luca, Giuseppe Molinari, Stefano Pulcini, Thelma A. Pertinhez, Erminia Di Bartolomeo, Rino Biguzzi, Roberto Baricchi, Chiara Marraccini
Summary: This study compared two methods for preparing optimal P-RBCs products for beta-thalassemia major patients, finding that method 1 resulted in higher RBC count and Hb content, while method 2 produced P-RBCs with lower levels of K+, iron, and storage lesions markers. Further clinical studies are needed to determine the impact of these differences on transfusion-related complications and the quality of life of beta TM patients.
Article
Biochemistry & Molecular Biology
Alkmini T. Anastasiadi, Efthymios C. Paronis, Vasiliki-Zoi Arvaniti, Athanasios D. Velentzas, Anastasia C. Apostolidou, Evangelos G. Balafas, Monika Dzieciatkowska, Nikolaos G. Kostomitsopoulos, Konstantinos Stamoulis, Issidora S. Papassideri, Angelo D'Alessandro, Anastasios G. Kriebardis, Marianna H. Antonelou, Vassilis L. Tzounakas
Summary: Blood donors with beta-thalassemia trait (beta Thal(+)) have shown to have good storage capabilities for red blood cells, which are resistant to lysis and oxidative/proteotoxic stress. These RBCs perform better post-storage and demonstrate higher recovery rates in animal models.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Multidisciplinary Sciences
Md Jubayer Hossain, Md Wahidul Islam, Ummi Rukaiya Munni, Rubaiya Gulshan, Sumaiya Akter Mukta, Md Sharif Miah, Sabia Sultana, Mousumi Karmakar, Jannatul Ferdous, Mohammad Ariful Islam
Summary: This study aimed to determine the health-related quality of life (HRQoL) and its determinants among thalassemia patients in Bangladesh. A cross-sectional survey was conducted on 356 randomly selected patients. Findings showed that male patients had higher scores in bodily pains and physical health summaries compared to female patients. Lower income, frequent blood transfusion, disease severity, comorbidities, and medical expenses were significantly associated with lower SF-36 scores.
SCIENTIFIC REPORTS
(2023)
Review
Biochemistry & Molecular Biology
Pei-Chin Lin, Wan-Yi Hsu, Po-Yi Lee, Shih-Hsien Hsu, Shyh-Shin Chiou
Summary: Thalassemia is a common congenital hemoglobinopathy with increasing incidence in various regions due to immigration. Hepatocellular carcinoma (HCC) is one of the major complications in patients with thalassemia, and its development is attributed to iron overload. Regular surveillance for HCC development is crucial in thalassemia patients, and the treatment approaches are similar to those in the general population. This article also reviews supplemental therapies and novel treatments for HCC in thalassemia patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Oncology
Volkan Hazar, Gulsun Tezcan Karasu, Vedat Uygun, Namik Ozbek, Musa Karakukcu, Gulyuz Ozturk, Hayriye Daloglu, Suar Caki Kilic, Tekin Aksu, Ekrem Unal, Ulker Kocak, Akif Yesilipek, Arzu Akcay, Orhan Gursel, Alphan Kupesiz, Fatma Visal Okur, Talia Ileri, Savas Kansoy, Ibrahim Bayram, Barbaros Sahin Karagun, Muge Gokce, Zuhre Kaya, Ikbal Ok Bozkaya, Turkan Patiroglu, Serap Aksoylar
LEUKEMIA & LYMPHOMA
(2020)
Article
Hematology
Nergiz Oner, Turkiz Gursel, Zuhre Kaya, Ebru Yilmaz Keskin, Ulker Kocak, Meryem Albayrak, Idil Yenicesu, Burcu Belen Apak, Melek Isik
TRANSFUSION AND APHERESIS SCIENCE
(2020)
Article
Hematology
Ayca Koca Yozgat, Goksel Leblebisatan, Sinan Akbayram, Simge Cinar Ozel, Zeynep Karakas, Erol Erduran, Sebnem Yilmaz, Ulker Kocak, Sule Unal, Gul Nihal Ozdemir, Meryem Albayrak, Emine Zengin, Yesim Oymak, Ozcan Bor, Hasan Fatih Cakmakli, Murat Soker, Dilek Gurlek Gokcebay, Huseyin Tokgoz, Baris Malbora, Serap Karaman, Tiraje Celkan, Ilgen Sasmaz, Nese Yarali, Hale Oren, Aysegul Unuvar, Namik Yasar Ozbek
TURKISH JOURNAL OF HEMATOLOGY
(2020)
Article
Oncology
Raya Saab, Anas Obeid, Fatiha Gachi, Houda Boudiaf, Lilit Sargsyan, Khulood Al-Saad, Tamar Javakhadze, Azim Mehrvar, Sawsan Sati Abbas, Yasir Saadoon Abed Al-Agele, Salma Al-Haddad, Mouroge Hashim Al Ani, Suleiman Al-Sweedan, Amani Al Kofide, Wasil Jastaniah, Nisreen Khalifa, Elie Bechara, Malek Baassiri, Peter Noun, Jamila El-Houdzi, Mohammed Khattab, Krishna Sagar Sharma, Yasser Wali, Naureen Mushtaq, Aliya Batool, Mahwish Faizan, Muhammad Rafie Raza, Mohammad Najajreh, Mohammed Awad Mohammed Abdallah, Ghada Sousan, Khaled M. Ghanem, Ulker Kocak, Tezer Kutluk, Hac Ahmet Demir, Hamoud Hodeish, Samar Muwakkit, Asim Belgaumi, Abdul-Hakim Al-Rawas, Sima Jeha
Article
Pediatrics
Volkan Hazar, Gulsun Tezcan Karasu, Gulyuz ozturk, Alphan Kupesiz, Serap Aksoylar, Namik ozbek, Vedat Uygun, Talia Ileri, Fatma Visal Okur, Ulker Kocak, Suar caki Kilic, Arzu Akcay, Elif Guler, Savas Kansoy, Musa Karakukcu, Ibrahim Bayram, Tekin Aksu, Akif Yesilipek, Barbaros Sahin Karagun, Sebnem Yilmaz, Mehmet Ertem, Duygu Uckan, Tunc Fisgin, Orhan Gursel, Yontem Yaman, Ceyhun Bozkurt, Muge Gokce
Summary: The study analyzed 255 children with relapse of acute leukemia after their first allo-HSCT, finding that isolated extramedullary relapse and relapse within 365 days post-transplantation were significant prognostic factors. The majority of children received salvage treatment with curative intent, with only a minority undergoing a second allo-HSCT.
PEDIATRIC TRANSPLANTATION
(2021)
Article
Hematology
Zuhre Kaya, Melek Isik, Nihan Oruklu, Serap Kirkiz, Emin Umit Bagriacik, Luis M. Allende, Maria J. Diaz-Madronero, Raquel Ruiz-Garcia, Faruk Guclu Pinarli, Pinar Gocun Uyar, Ulker Kocak
Summary: This study found that ALPS may not be rare in certain groups of children, with predictive factors including anemia, thrombocytopenia, splenomegaly, lymphadenopathy, and young age. Screening for ALPS is recommended in children with lymphoma presenting with cytopenia, those with nonmalignant organomegaly presenting with immune cytopenia, and those with chronic immune thrombocytopenic purpura or autoimmune hemolytic anemia developing organomegaly during follow-up.
TURKISH JOURNAL OF HEMATOLOGY
(2021)
Article
Oncology
Tayyibe Sever, Zuhre Kaya, Serap Kirkiz, Ali Celik, Ismail Akdulum, Tugba R. Gursoy, Ayse T. Aslan, Melis Deniz, Anil Tapisiz, Ulker Kocak
Summary: Thoracic air leak syndromes (TALS) are rare pulmonary complications that can have various risk factors. This report presents the case of a 14-year-old girl who developed multiple complications following allo-HSCT. This is the first report of a child developing these subsequent complications after allo-HSCT. Therefore, the manifestations and management options for these unfamiliar complications are discussed.
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
(2022)
Article
Oncology
Ozlem Tufekci, Melike Sezgin Evim, Adalet Meral Gunes, Tiraje Celkan, Deniz Yilmaz Karapinar, Zuhre Kaya, Birsen Baysal, Birol Baytan, Ulker Kocak, Sebnem Yilmaz, Suzan Cinar, Hale Oren
Summary: Assessing minimal residual disease (MRD) is crucial for risk classification and personalized therapy in childhood acute lymphoblastic leukemia (ALL). A study in Turkey retrospectively collected data of pediatric ALL patients treated with Berlin-Frankfurt-Munster (BFM) protocols. MRD assessment was done using real-time quantitative polymerase chain reaction (qPCR) and multiparametric flow cytometry (MFC). MRD monitoring led to upgrading 2% of patients from intermediate risk group to high-risk group. Factors such as age ≥10 years, poor response to prednisone, PCR-MRD ≥10(-3) on day 33 and day 78 were identified as poor prognostic factors affecting event-free survival (EFS). Establishing infrastructure and ensuring standardization for MRD methods are crucial for optimal management of children with ALL.
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
(2022)
Letter
Oncology
Sirma Karamercan, Zuhre Kaya, Ulker Kocak
PEDIATRIC BLOOD & CANCER
(2022)
Review
Hematology
Tayyibe Sever, Serap Kirkiz, Zuhre Kaya, Ulker Kocak
Summary: Minor ABO mismatch is associated with the development of acute GVHD, whereas major and bidirectional ABO incompatibility, as well as blood component transfusions, do not contribute to aGVHD development in children undergoing HSCT with myeloablative conditioning.
TRANSFUSION AND APHERESIS SCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Zuhre Kaya, Deniz Yuce, Serap Kirkiz, Ulker Kocak, Fusun Ozmen
Summary: This study is the first to investigate the prognostic role of cytokines and sHLA-G levels in pediatric leukemia patients who have undergone allo-SCT. The study found that the levels of all cytokines tested and sHLA-G were significantly higher at diagnosis and at the post-transplant time points compared to the pre-transplantation period. Elevated IL-4, IL-10, and/or sHLA-G at diagnosis and post-transplantation (specifically at day 14) were associated with higher relapse rates, while elevated IL-2 and TNF-alpha at the time of transplantation were associated with lower survival rates. Serum cytokines and sHLA-G levels may serve as useful predictors of survival and relapse in these patients.
Article
Medicine, General & Internal
Elif Keles Gulnerman, Yagmur Cam, Bulent Elbasan, Sebnem Soysal, Zuhre Kaya, Idil Yenicesu, Ulker Kocak
Summary: The study finds that there is no significant correlation between the time after treatment and the quality of life for childhood ALL survivors, but there is a significant association between physical capacities and daily independent living status. Reading, writing, and mathematical skills are significantly associated with the duration since completion of therapy.
TURKISH JOURNAL OF MEDICAL SCIENCES
(2021)
Article
Hematology
Volkan Hazar, Gulyuz Ozturk, Koray Yalcin, Vedat Uygun, Serap Aksoylar, A. Kupesiz, Ikbal Ok Bozkaya, Barbaros Sahin Karagun, Ceyhun Bozkurt, Talia Ileri, Didem Atay, Ulker Kocak, Gulsun Tezcan Karasu, Akif Yesilipek, Muge Gokce, Savas Kansoy, Gulen Tuysuz Kintrup, Musa Karakukcu, Fatma Visal Okur, Mehmet Ertem, Zuhre Kaya, Orhan Gursel, Yontem Yaman, Namik Ozbek, Bulent Antmen, Ozlem Tufekci, Canan Albayrak, Basak Adakli Aksoy, Gulay Sezgin, Davut Albayrak, Melike Sezgin Evim, Emine Zengin, Esra Pekpak
Summary: Relapse after allo-HSCT remains a common cause of post-transplantation mortality. Isolated extramedullary relapse (iEMR) is relatively rare, with CR2+/active disease at transplantation and prior EM disease as independent risk factors for iEMR. The prognosis of iEMR is poor, especially after a second relapse, highlighting the need for improved strategies to enhance outcomes.
TRANSPLANTATION AND CELLULAR THERAPY
(2021)
Article
Pediatrics
Nurettin Erdem, Tugba Bedir Demirdag, Hasan Tezer, Burcu Ceylan Cura Yayla, Fatma Nur Baran Aksakal, Anil Taptstz, Oksan Derinoz, Arzu Okur, Faruk Guclu Pinarli, Ulker Kocak, Aysun Bideci
Summary: This study evaluated the performance of different methods of measuring body temperature in children, with results showing that axillary thermometers can lead to increased temperature up to 8 minutes after placement, non-contact skin thermometers should be used only for follow-up of fever patients, and tympanic and axillary thermometers may be preferable for initial fever diagnosis.
TURKISH JOURNAL OF PEDIATRICS
(2021)
Article
Medicine, General & Internal
Soner Sertan Kara, Hasan Tezer, Meltem Polat, Urcu Ceylan Cura Yayla, Tugba Bedir Demirdag, Arzu Okur, Ali Fettah, Saliha Kanik Yuksek, Anil Tapisiz, Zuhre Kaya, Namik Ozbek, Idil Yenicesun, Nese Yarali, Ulker Kocak
TURKISH JOURNAL OF MEDICAL SCIENCES
(2019)
