Review
Immunology
Aurora Chinnici, Linda Beneforti, Francesco Pegoraro, Irene Trambusti, Annalisa Tondo, Claudio Favre, Maria Luisa Coniglio, Elena Sieni
Summary: Hemophagocytic Lymphohistiocytosis (HLH) is a rare clinical condition characterized by sustained but ineffective immune system activation, leading to severe and systemic hyperinflammation. Prompt diagnosis and treatment are crucial for survival, as a considerable proportion of patients with HLH still die from progressive disease. Expert consultation and genetic analysis are recommended for accurate interpretation and therapeutic decisions.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medical Laboratory Technology
Tanya Sajan Ponnatt, Cullen M. Lilley, Kamran M. Mirza
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of immune regulation that requires rapid diagnosis and aggressive management. Understanding the pathogenesis and early diagnosis of HLH plays a crucial role in determining patient outcome. HLH can be caused by genetic mutations or acquired factors, and proper management is essential for improving prognosis.
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
(2022)
Review
Medicine, Research & Experimental
Chiara Benevenuta, Ilaria Mussinatto, Cecilia Orsi, Fabio S. S. Timeus
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by uncontrolled and ineffective immune response, leading to hyperinflammation. This review focuses on secondary HLH in pediatric patients, discussing its epidemiology, pathophysiology, diagnosis, treatment, prognosis, as well as subtypes and new insights in diagnosis and treatment.
EXPERIMENTAL AND THERAPEUTIC MEDICINE
(2023)
Article
Immunology
Deli Song, Jingshi Wang, Jia Zhang, Junxia Hu, Chaofan Wu, Zhao Wang
Summary: In this article, five cases of HAVCR2 mutation-associated HLH were reported. The study found an elevated level of IL-1RA in the serum of these patients. The potential mechanisms underlying HLH associated with HAVCR2 mutation were also investigated.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Hematology
Wenxin Ou, Yunze Zhao, Ang Wei, Honghao Ma, Qing Zhang, Liping Zhang, Hongyun Lian, Dong Wang, Zhigang Li, Tianyou Wang, Rui Zhang
Summary: This study compared the serum levels of 34 cytokines in children with HLH and explored the specific cytokine pattern of different HLH subtypes. The levels of IFN-gamma and IL-18 were elevated in more than 90% of patients, while other cytokines showed varying levels of increase. Different HLH subtypes exhibited distinct cytokine patterns, and the ratio of cytokines was more significant in differentiating subtypes. Elevated GM-CSF and MCP-1 were identified as independent risk factors for poor prognosis in HLH patients. Various cytokines play important roles in HLH and can be useful biomarkers for prognosis.
ANNALS OF HEMATOLOGY
(2023)
Article
Immunology
Bishara J. Freij, Aidan T. Hanrath, Rui Chen, Sophie Hambleton, Christopher J. A. Duncan
Summary: This study identified a novel genetic cause of homozygous STAT2 deficiency with unique clinical features, including HLH, severe influenza, and vaccine-strain varicella. Genetic investigation revealed a novel nonsense STAT2 variant that led to a lack of STAT2 protein expression and impaired IFN-I signaling. The occurrence of life-threatening influenza and vaccine-strain varicella in association with STAT2 deficiency highlights the critical role of IFN-I/III immunity in viral infections and vaccine responses.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Biology
Anshul Vagrecha, Mingce Zhang, Suchitra Acharya, Shannon Lozinsky, Aaron Singer, Chana Levine, Maha Al-Ghafry, Carolyn Fein Levy, Randy Q. Cron
Summary: Children with COVID-19 have the potential to develop a novel syndrome known as MIS-C, characterized by high levels of inflammation. This study discovered that a small number of children with MIS-C carried genetic mutations, including a variant called DOCK8, which may be a risk factor for the development of this syndrome. These findings contribute to a better understanding of the mechanisms underlying MIS-C.
Letter
Oncology
Liang V. Tang, Yu Hu
Summary: This is the first reported case of a healthy individual developing hemophagocytic lymphohistiocytosis (HLH) after receiving the COVID-19 vaccine. The patient's symptoms gradually improved after treatment. The study suggests that excluding the presence of active EBV infection or other common viruses before COVID-19 vaccination is crucial.
JOURNAL OF HEMATOLOGY & ONCOLOGY
(2021)
Article
Immunology
Yue Song, Xiaoli Li, Xuefeng He, Fei Zhou, Feng Du, Ziyan Wang, Suning Chen, Depei Wu
Summary: This study investigates the use of dose-escalating ruxolitinib in HLH patients who did not respond to the general dose. The results show that half of the patients achieved better remission after dose escalation, and sCD25 levels can serve as an early indicator for considering chemotherapy during treatment.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Multidisciplinary Sciences
Xin Luo, Chentao Zhou, Cunwei Ji, Chunmin Lu, Yasha Luo, Zhenhui Chen, Tianhua Zhong, Ruoting Ye, Liwei Zeng, Mingyong Luo
Summary: HLH is a rare but fatal complication in children with sepsis, characterized by elevated levels of enzymes, lipids, and ferritin, as well as decreased neutrophils, hemoglobin, and other indicators. Other symptoms include prolonged fever, hepatomegaly, and splenomegaly. Hypofibrinogenemia is an independent predictor for HLH in children with sepsis, with a cutoff value of <2.43 g/L.
SCIENTIFIC REPORTS
(2023)
Review
Hematology
Chenzi Zhao, Qing Zhang, Rui Zhang, Hongyun Lian, Honghao Ma, Xiaoxi Zhao, Zhigang Li
Summary: This study analyzed the genetic variation and prognosis of primary hemophagocytic lymphohistiocytosis (pHLH) in children and the clinical features of isolated central nervous system HLH (CNS-HLH). The most common gene variants in pHLH patients were UNC13D, PRF1, and XIAP. CNS involvement was higher in pHLH patients compared to secondary HLH. Isolated CNS-HLH patients are often misdiagnosed, and hematopoietic stem cell transplantation (HSCT) may be beneficial for these patients. NK cell cytotoxicity and CD107a levels were not accurate in distinguishing pHLH from secondary HLH.
ANNALS OF HEMATOLOGY
(2023)
Article
Pediatrics
Li Xiao, Ximing Xu, Zhiling Zhang, Ying Dou, Xianmin Guan, Yuxia Guo, Jie Yu
Summary: This study retrospectively investigated the association between serum cholesterol levels and prognosis in pediatric HLH patients. The results showed that decreased total cholesterol level is an independent risk factor for early death within 30 days after diagnosis.
FRONTIERS IN PEDIATRICS
(2023)
Article
Immunology
Zhiyu Zhang, Junqian Liu, Jingyue Wang, Yushi Wang
Summary: This case analysis discusses a 73-year-old Chinese man admitted to the ICU with Klebsiella pneumonia-induced HLH, treated successfully with a combination of meropenem and dexamethasone. It is recommended that healthcare teams pay attention to the role of hyperferritinemia in diagnosing HLH and include ferritin in their monitoring protocols in the ICU.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Immunology
Erica A. Steen, Michelle L. Hermiston, Kim E. Nichols, Lauren K. Meyer
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disorder caused by mutations in genes necessary for cytotoxic granule assembly and function, leading to ineffective immune responses. The resulting persistent immune system stimulation drives severe tissue inflammation and end-organ damage. Evidence supports a potential digenic mode of inheritance, where mutations in two different genes cooperate to impair pathway activity.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Stephanie J. Si, Sarah K. Tasian, Hamid Bassiri, Brian T. Fisher, Jasmyn Atalla, Reema Patel, Neil Romberg, Michele P. Lambert, Michele Paessler, Edward J. Behrens, David T. Teachey, Kathleen E. Sullivan
Summary: HLH is a syndrome of severe immune dysregulation with a complex diagnosis process, leading to potential delays in treatment initiation.
JOURNAL OF CLINICAL IMMUNOLOGY
(2021)
Article
Pediatrics
Emrah Gun, Tanil Kendirli, Edin Botan, Nazmiye Turker, Anar Gurbanov, Burak Balaban, Ali Genco Gencay, Gul Arga, Selen Karagozlu, Mehmet Gokhan Ramoglu, Halil Ozdemir, Tayfun Ucar, Ercan Tutar, Ergin Ciftci
Summary: Multisystem inflammatory syndrome in children (MIS-C) can manifest with various clinical features and can lead to life-threatening critical illness. Most children require intensive care and immunomodulation treatment with favorable outcomes.
JOURNAL OF PEDIATRIC INTENSIVE CARE
(2023)
Article
Cardiac & Cardiovascular Systems
Mehmet G. Ramoglu, Selen Karagozlu, Ozlem Bayram, Jeyhun Bakhtiyarzada, Alperen Aydin, Goksel Vatansever, Halil Ozdemir, Deniz Tekin, Tayfun Ucar, Ergin Ciftci, Ercan Tutar
Summary: Routine troponin screening does not provide much information in previously healthy pediatric COVID-19 patients without any signs of myocardial dysfunction. Elevated troponin levels may be observed, but it is mostly a sign of myocardial injury without detectable myocardial dysfunction in this group of patients.
CARDIOLOGY IN THE YOUNG
(2022)
Article
Pediatrics
Dilek Yilmaz Ciftdogan, Yildiz Ekemen Keles, Benhur Sirvan Cetin, Nazan Dalgic Karabulut, Melike Emiroglu, Zafer Bagci, Ayse Buyukcam, Emine Hafize Erdeniz, Gul Arga, Edanur Yesil, Ozlem Cakici, Adem Karbuz, Zumrut Sahbudak Bal, Soner Sertan Kara, Arife Ozer, Ozge Metin Akcan, Sefika Elmas Bozdemir, Ayse Berna Anil, Hatice Uygun, Omer Kilic, Selda Hancerli Torun, Zuhal Umit, Murat Sutcu, Berfin Ozgokce Ozmen, Hatice Karaoglu Asrak, Gulsum Alkan, Ahu Kara Aksay, Cuneyt Ugur, Ahmet Ziya Birbilen, Burcu Bursal Duramaz, Esra Akyuz Ozkan, Ozgur Burakay, Sema Yildirim Arslan, Eda Karadag Oncel, Serkan Fazli Celik, Ahmet Osman Kilic, Seval Ozen, Remzi Sarikaya, Demet Demirkol, Gazi Arslan, Ozden Turel, Ahmet Sert, Ergul Sari, Zerrin Orbak, Irfan Oguz Sahin, Celal Varan, Hacer Akturk, Sadiye Kubra Tuter Oz, Fatih Durak, Mehmet Burhan Oflaz, Manolya Kara, Derya Karpuz, Mey Talip Petmezci, Nevin Hatipoglu, Selim Oncel, Mehmet Turgut, Ferhan Elmali, Ayper Somer, Necdet Kuyucu, Ener Cagri Dinleyici, Zafer Kurugol, Ergin Ciftci, Ates Kara
Summary: This study retrospectively analyzed the clinical and laboratory features of MIS-C patients with or without overlap with Kawasaki disease (KD). The findings showed that almost half of the MIS-C patients had clinical features that overlapped with KD, especially incomplete KD. Patients with overlap with KD had lower lymphocyte and platelet counts, and higher levels of ferritin and procalcitonin.
EUROPEAN JOURNAL OF PEDIATRICS
(2022)
Article
Infectious Diseases
Seval Ozen, Halil Ozdemir, Ebru Evren, Esra Cakmak Taskin, Gul Arga, Hatice Kubra Konca, Hasan Fatih Cakmakli, Sule Haskologlu, Emel Okulu, Handan Dincaslan, Elif Ince, Talia Ileri, Nurdan Tacyildiz, Figen Dogu, Ebru Us, Zeynep Ceren Karahan, Suat Fitoz, Tanil Kendirli, Zarife Kuloglu, Ercan Tutar, Aydan Ikinciogullari, Emel Unal, Mehmet Ertem, Erdal Ince, Ergin Ciftci
Summary: The study investigated the utility of GM-EIA as a diagnostic tool for IA in pediatric patients, finding that it can be used for both screening and diagnostic purposes at a cut-off value of >= 1.5.
INFECTIOUS DISEASES
(2022)
Article
Infectious Diseases
Gul Arga, Hatice Kubra Konca, Ayca Nur Celik, Edin Botan, Utku Caglayan, Halil Ozdemir, Goksel Vatansever, Tanil Kendirli, Ergin Ciftci
Summary: This study reports the first case in Turkey of invasive meningococcal infection caused by serogroup Z of Neisseria meningitidis. The current vaccines do not cover the Z serogroup, highlighting the need for continued disease surveillance and implementation of effective prevention and control strategies for nonvaccine serogroups.
JOURNAL OF PEDIATRIC INFECTIOUS DISEASES
(2022)
Editorial Material
Pediatrics
Ergin Ciftci, Tanil Kendirli
JOURNAL OF PEDIATRIC INFECTION
(2022)
Article
Pediatrics
Tugba Erat, Halil Ozdemir, Aysun Yahsi, Tugce Tural Kara, Ebru Azapagasi, Tanil Kendirli, Elif Ince, Begum Atasay, Emel Unal, Derya Aysev, Erdal Ince, Ergin Ciftci
Summary: This study retrospectively examined the epidemiological characteristics of Serratia marcescens in a children's hospital in Turkey. The results showed that S. marcescens isolates exhibited resistance to polymyxins and produced ESBL and carbapenemase. High-dose prolonged-infusion of meropenem and early catheter removal were found to reduce the mortality rate of S. marcescens infection.
JOURNAL OF PEDIATRIC INFECTION
(2022)
Article
Microbiology
Seda Hancerli Demirbas, Halil Ozdemir, Sukriye Yilmaz, Gul Arga, Hatice Kubra Konca, Zeynep Ceren Karahan, Suat Fitoz, Ergin Ciftci
Summary: The current study aimed to investigate the clinical, laboratory and radiological findings of children with pneumonia confirmed as M.pneumoniae, and to determine the factors for diagnosis. The study included 77 children, with a median age of 31 months. The most common symptoms were cough and fever.
MIKROBIYOLOJI BULTENI
(2023)
Article
Pediatrics
Tugba Erat, Aysun Yahsi, TugceTural Kara, Halil Ozdemir, Ahmet Derya Aysev, Ergin Ciftci, Erdal Ince
Summary: This report defines Stenotrophomonas maltophilia-associated pseudobacteraemia as a result of contamination of the syringe tip with citrated coagulation tubes without blood culture cultivation. Environmental culture samples were taken to find the source of pseudobacteraemia and S. maltophilia was not found in any of them. Blood cultures taken simultaneously with the blood culture were questioned, and cultures were taken from the test tubes. S. maltophilia with the same antibiogram as trimethoprim sulfamethoxazole resistant fluoroquinolone susceptible was isolated from blood culture growths in all citrated coagulation tubes.
JOURNAL OF PEDIATRIC INFECTION
(2022)
Editorial Material
Pediatrics
Ergin Ciftci, Hatice Belkis Inceli, Halil Ozdemir, Sule Haskologlu, Figen Dogu
JOURNAL OF PEDIATRIC INFECTION
(2022)
Article
Infectious Diseases
Merve Havan, Tanil Kendirli, Ozgun Tutku Parlar, Serhan Ozcan, Aysun Yahsi, Tugba Erat, Duygu Ocal, Haluk Guriz, Halil Ozdemir, Zeynep Ceren Karahan, Ergin Ciftci, Erdal Ince
Summary: This study shares the experience of treating OXA-48-positive PDR-CRKP infection in a pediatric intensive care unit, which was classified as a clinical outbreak. The outbreak was successfully managed with combination antibiotic therapy and strict adherence to infection control measures, resulting in no treatment-related complications and a mortality rate of 9%.
MICROBIAL DRUG RESISTANCE
(2023)
Editorial Material
Pediatrics
Ergin Ciftci
JOURNAL OF PEDIATRIC INFECTION
(2023)
Article
Oncology
Merve Cikili-Uytun, Mehtap Eroglu, Mehmet Ertem, Dilber Talia Ileri, Elif Ince, Birim Guenay Kilic
Summary: This study aimed to investigate the psychiatric problems, health-related quality of life (HRQoL), and self-esteem scores of thalassemia patients who underwent hematopoietic stem cell transplantation (HSCT). The results showed that HSCT patients had higher HRQoL and self-esteem scores compared to thalassemia patients, but both groups had a high prevalence of mental disorders.
PEDIATRIC HEMATOLOGY AND ONCOLOGY
(2023)
Letter
Pediatrics
Anar Gurbanov, Tanil Kendirli, Edin Botan, Fevzi Kahveci, Ali Genco Gencay, Gul Arga, Halil Ozdemir, Ergin Ciftci
TURKISH ARCHIVES OF PEDIATRICS
(2022)
