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)
Article
Immunology
Sylvain Raimbault, Guillaume Monneret, Morgane Gossez, Fabienne Venet, Alexandre Belot, Franck Zekre, Solene Remy, Etienne Javouhey
Summary: In this study, the researchers examined the use of monocyte human leucocyte antigen (mHLA)-DR as a potential diagnostic marker for secondary HLH (sHLH). They found that levels of mHLA-DR were significantly higher in patients with sHLH compared to healthy children and those with septic shock. Additionally, mHLA-DR levels decreased after treatment initiation. The findings suggest that elevated mHLA-DR could aid in the diagnosis of sHLH and distinguish it from septic shock.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Infectious Diseases
Marie-Lisa Hieber, Rosanne Sprute, Dennis A. Eichenauer, Michael Hallek, Ron D. Jachimowicz
Summary: This study reported a case of severe HLH after SARS-CoV-2 immunization and reviewed all reported cases of COVID-19 vaccine-associated HLH. It was found that COVID-19 vaccines may occasionally trigger HLH, and Anakinra may be an efficacious treatment option for this condition.
Article
Virology
Hans Martin Orth, Andre Fuchs, Nadine Luebke, Bjoern-Erik Ole Jensen, Tom Luedde
Summary: We report an unusual case of recurrent Hemophagocytic lymphohistiocytosis (HLH) caused by Parvovirus B19 infection. The recurrent viremia triggered three episodes of hyperinflammation, two of which were clearly diagnosed as HLH. This case provides new insight into triggering mechanisms for HLH.
Article
Immunology
Antonia Pascarella, Claudia Bracaglia, Ivan Caiello, Alessia Arduini, Gian Marco Moneta, Marianna Nicoletta Rossi, Valentina Matteo, Manuela Pardeo, Fabrizio De Benedetti, Giusi Prencipe
Summary: This study found that untreated sHLH/MAS patients had higher levels of phosphorylated STAT1 in monocytes compared to glucocorticoid-treated patients, indicating a hyperreactivity to IFN gamma stimulation. These findings suggest that pSTAT1 levels may serve as a potential biomarker for early identification of sHLH/MAS.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Engineering, Biomedical
Honglan Wang, Huiwen Liu, Jia Li, Chunying Liu, Hui Chen, Junying Li, Chunyan Sun, Tao Guo, Zhiqing Pang, Bo Zhang, Yu Hu
Summary: LMNP, a novel type of macrophage membrane-coated nanoparticles, can effectively dampen HLH and alleviate related symptoms. This study provides alternative insights for the treatment of other cytokine storm-mediated pathologic conditions.
BIOACTIVE MATERIALS
(2023)
Article
Medicine, General & Internal
Sebastian Schnaubelt, Daniel Tihanyi, Robert Strassl, Ralf Schmidt, Sonja Anders, Anton N. Laggner, Hermine Agis, Hans Domanovits
Summary: The study reviewed three patients with COVID-19 who exhibited hyperinflammation and cytokine storm, requiring intensive care treatment. Diagnosis of secondary sHLH was made using HScore. A treatment regimen of methylprednisolone, pentaglobin, and anakinra was developed and administered. One patient survived while two others, diagnosed late, deceased.
Review
Oncology
Jonathan Paolino, Nancy Berliner, Barbara Degar
Summary: HLH is a syndrome of multiorgan system dysfunction caused by hypercytokinemia and persistent activation of cytotoxic T lymphocytes and macrophages. A common finding and diagnostic criterion of HLH is cytopenias in >= 2 cell lines. The mechanism of cytopenias in HLH is multifactorial, mainly driven by suppression of hematopoiesis by pro-inflammatory cytokines.
FRONTIERS IN ONCOLOGY
(2022)
Article
Medicine, General & Internal
Halil Yildiz, Diego Castanares-Zapatero, Philippe d'Abadie, Sarah Bailly, Jean Cyr Yombi
Summary: This study examined the clinical and biological characteristics of adult HLH patients in Belgium, revealing that malignancy and infection were the most common triggers. Risk factors for mortality included age, male sex, malignancy, and DIC, with age being the only significant factor in multivariate analysis.
INTERNATIONAL JOURNAL OF GENERAL MEDICINE
(2022)
Article
Immunology
David X. Liu, Bapi Pahar, Timothy K. Cooper, Donna L. Perry, Huanbin Xu, Louis M. Huzella, Ricky D. Adams, Amanda M. W. Hischak, Randy J. Hart, Rebecca Bernbaum, Deja Rivera, Scott Anthony, Marisa St Claire, Russell Byrum, Kurt Cooper, Rebecca Reeder, Jonathan Kurtz, Kyra Hadley, Jiro Wada, Ian Crozier, Gabriella Worwa, Richard S. Bennett, Travis Warren, Michael R. Holbrook, Connie S. Schmaljohn, Lisa E. Hensley
Summary: In this study, the researchers found that rhesus monkeys infected with the Ebola virus exhibit pathophysiological features similar to hemophagocytic lymphohistiocytosis syndrome/macrophage activation syndrome. Therefore, regulating inflammation and immune function may provide an effective treatment for controlling the pathogenesis of acute Ebola virus disease.
JOURNAL OF INFECTIOUS DISEASES
(2023)
Review
Gastroenterology & Hepatology
Elliot S. Coburn, Corey A. Siegel, Michael Winter, Eric D. Shah
Summary: HLH is rare among IBD patients treated with biologics, with most cases having identifiable infection or malignancy at diagnosis and a history of immunomodulator use. Survival rate is 70%, with risk factors possibly including younger age, male gender, Crohn's disease, and treatment induction phase.
DIGESTIVE DISEASES AND SCIENCES
(2021)
Article
Biology
Daniel D. Reiff, Mingce Zhang, Randy Q. Cron
Summary: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by uncontrolled inflammation and immune system activation. Primary HLH (pHLH) is caused by genetic mutations that lead to organ failure and can be fatal. Secondary HLH (sHLH) can be triggered by genetic mutations in certain genes. This study focused on the significance of a DOCK2 mutation in the pathogenesis of sHLH and its implications for diagnosis and treatment.
Article
Pediatrics
Oksana Boyarchuk, Olha Dyvonyak, Tetyana Hariyan, Alla Volokha
Summary: This article reports a 3-year-old boy with APECED who had recurrent COVID-19 and developed retinopathy and autoimmune hepatitis after the first episode of SARS-CoV-2 infection. Subsequent infections triggered severe hyperinflammation with signs of HLH. Despite treatment, the patient had a fatal outcome. This article highlights the importance of recognizing and managing HLH in patients with immune dysregulation and impaired viral response.
FRONTIERS IN PEDIATRICS
(2023)
Article
Rheumatology
S. D. Good, S. D. Wade, V. C. Kyttaris
Summary: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening inflammatory syndrome that can be triggered by autoimmune diseases, malignancy, or infection. This study aimed to compare the clinical features and laboratory parameters of macrophage activation syndrome (MAS) and malignancy-associated HLH (mHLH) patients. Significant differences were found in platelet counts, soluble IL-2R concentration, and hepatomegaly between the two groups.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Rheumatology
Maria L. Taylor, Kacie J. Hoyt, Joseph Han, Leslie Benson, Siobhan Case, Mia T. Chandler, Margaret H. Chang, Craig Platt, Ezra M. Cohen, Megan Day-Lewis, Fatma Dedeoglu, Mark Gorman, Jonathan S. Hausmann, Erin Janssen, Pui Y. Lee, Jeffrey Lo, Gregory P. Priebe, Mindy S. Lo, Esra Meidan, Peter A. Nigrovic, Jordan E. Roberts, Mary Beth F. Son, Robert P. Sundel, Maria Alfieri, Jenny Chan Yeun, Damilola M. Shobiye, Barbara Degar, Joyce C. Chang, Olha Halyabar, Melissa M. Hazen, Lauren A. Henderson
Summary: Implementing an evidence-based guideline in the management of hemophagocytic lymphohistiocytosis and macrophage activation syndrome in children improves clinical outcomes, including reduction in mortality, shorter time to decrease in inflammatory markers, and improved treatment effects.
JOURNAL OF RHEUMATOLOGY
(2022)
Article
Pharmacology & Pharmacy
Iwona E. Glowacka, Katarzyna Gawron, Dorota G. Piotrowska, Mirthe Graus, Graciela Andrei, Dominique Schols, Robert Snoeck, Anita Camps, Emiel Vanhulle, Kurt Vermeire
Summary: In this study, a series of 48 hybrids were synthesised and evaluated for antiviral activity. The hybrids contained a diethoxyphosphoryl group connected to the triazole moiety via ethylene or propylene linker, and substituted benzyl or benzoyl function in the quinazoline-2,4-dione moiety. The hybrids showed potent antiviral activities against Respiratory Syncytial Virus (RSV), with some weak activity against varicella zoster virus and human cytomegalovirus.
ANTIVIRAL RESEARCH
(2023)
Article
Pediatrics
Lien De Somer, Brigitte Bader-Meunier, Sylvain Breton, Sara Brachi, Carine Wouters, Francesco Zulian
Summary: This study retrospectively collected data on the clinical features, radiological manifestations, and disease course of dry synovitis (DS) patients and compared them with rheumatoid factor negative polyarticular juvenile idiopathic arthritis (RFneg-polyJIA) patients. The results showed that DS patients had a higher number of joint involvement, more severe joint contractures, and a longer diagnostic delay compared to RFneg-polyJIA patients.
PEDIATRIC RHEUMATOLOGY
(2023)
Article
Immunology
Karen Yu, Luna Dillemans, Mieke Gouwy, Helena Bessa, Mieke Metzemaekers, Erik Martens, Patrick Matthys, Xavier Bossuyt, Patrick Verschueren, Carine Wouters, Lien De Somer, Paul Proost
Summary: In this study, a fluorescent in vitro assay was improved to characterize endogenous PAD activity in complex samples. The results showed similar levels of PAD activity in synovial fluids of rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) patients, while citrullination was limited in joints of patients with gout or Lyme's disease. Interestingly, a higher level of extracellular citrullination was only found in blood of anti-CCP-positive RA patients.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Biochemistry & Molecular Biology
M. P. Paramonova, E. S. Gureeva, A. A. Ozerov, R. Snoeck, G. Andrei, D. A. Aleksandrov, A. L. Khandazhinskaya, M. S. Novikov, S. N. Kochetkov
Summary: A new series of 1-[ω-(bromophenoxy)alkyl]-uracil derivatives containing various fragments were synthesized and tested for their antiviral activity against human cytomegalovirus. It was found that the compound with a bridge of five methylene groups exhibited high anti-cytomegalovirus activity in vitro.
DOKLADY BIOCHEMISTRY AND BIOPHYSICS
(2023)
Correction
Biochemistry & Molecular Biology
Kourosh Ahmadzadeh, Marie Pereira, Margot Vanoppen, Eline Bernaerts, Jeong-Hun Ko, Tania Mitera, Christy Maksoudian, Bella B. Manshian, Stefaan Soenen, Carlos D. Rose, Patrick Matthys, Carine Wouters, Jacques Behmoaras
Article
Chemistry, Medicinal
Roberta Bivacqua, Marilia Barreca, Virginia Spano, Maria Valeria Raimondi, Isabella Romeo, Stefano Alcaro, Graciela Andrei, Paola Barraja, Alessandra Montalbano
Summary: Viruses are responsible for causing various pathological conditions, with efforts made to identify selective viral targets for developing efficient therapeutics. Polymerases are attractive targets due to their crucial role in the viral life cycle. This overview focuses on non-nucleoside triazole-based compounds discovered in the past fifteen years as potential viral polymerase inhibitors, highlighting the structure-activity relationships necessary for developing effective antiviral agents.
EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY
(2023)
Article
Allergy
Mathijs Willemsen, John S. Barber, Erika Van Nieuwenhove, Frederik Staels, Margaux Gerbaux, Julika Neumann, Teresa Prezzemolo, Emanuela Pasciuto, Vasiliki Lagou, Nancy Boeckx, Jessica Filtjens, Amber De Visscher, Patrick Matthys, Rik Schrijvers, Thomas Tousseyn, Mark O'Driscoll, Giorgia Bucciol, Susan Schlenner, Isabelle Meyts, Stephanie Humblet-Baron, Adrian Liston
Summary: This study reported a patient with severe congenital neutropenia and syndromic features, and a homozygous missense mutation in the DBF4 gene was identified using whole exome sequencing. This mutation caused defects in granulocyte differentiation and DNA replication, resulting in impaired immune system function.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2023)
Article
Immunology
Hanna Helena Schalkwijk, Aspasia Georgala, Sarah Gillemot, Arturo Temblador, Dimitri Topalis, Sebastian Wittnebel, Graciela Andrei, Robert Snoeck
Summary: This study conducted long-term follow-up of a patient with immunodeficiency before and after stem cell transplantation, revealing the evolution and frequent reactivation of herpes simplex virus 1 strains. The multidrug resistance phenotype of a novel DNA polymerase mutation was confirmed by gene editing. This research is important for understanding virus evolution and drug resistance mutations in immunodeficient patients.
JOURNAL OF INFECTIOUS DISEASES
(2023)
Letter
Medicine, General & Internal
Ellen De Langhe, Sien Van Loo, Bert Malengier-Devlies, Mieke Metzemaekers, Frederik Staels, Jessica Vandenhaute, Nathalie Berghen, Raf Sciot, Anniek Corveleyn, Olga Tsuiko, Mieke Gouwy, Jan Lenaerts, Patrick Verschueren, Carine H. Wouters, Paul Proost, Patrick Matthys, Eric Legius, Rik Schrijvers
Summary: The article reports a case of adult-onset inflammatory disease. It is revealed that two somatic genetic events are responsible for the disease and its onset in adulthood.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Rheumatology
Hermine Brunner, Nikolay Tzaribachev, Ingrid Louw, Inmaculada Calvo Penades, Francisco Avila-Zapata, Gerd Horneff, Ivan J. Foeldvari, Daniel Kingsbury, Maria Eliana Paz Gastanaga, Carine Wouters, Johannes Breedt, Robert Wong, Margarita Askelson, Joe Zhuo, Alberto J. Martini, Daniel Lovell, Nicolino Ruperto
Summary: This study aimed to investigate the frequency and trajectories of individual patients with polyarticular-course juvenile idiopathic arthritis (JIA) achieving various composite end points on abatacept treatment. The results showed that patients achieved composite end points of low disease activity (LDA) with minimal pain, LDA with Childhood Health Assessment Questionnaire disability index score of 0, and 50% improvement in American College of Rheumatology criteria for JIA (ACR50) with minimal pain at 4 months. The proportion of patients meeting these end points increased over 21 months of abatacept treatment.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Wafa Hamdi, Hanene Ferjani, Raffaella Carlomagno, Perrine Dusser, Stephane Echaubard, Alexandre Belot, Kenza Bouayed, Carine Wouters, Olivier Richer, Veronique Hentgen, Anne Lohse, Diana Dan, Daniela Kaiser, Catherine Barbier, Elvira Cannizzaro, Sylvaine Poignant, Isabelle Melki, Michael Hofer
Summary: This study aims to identify the factors associated with poor prognosis of hip involvement in children with juvenile idiopathic arthritis (JIA) and evaluate treatment response. Male gender, enthesitis-related arthritis, and North African origin were found to be factors associated with hip arthritis. Hip inflammation was associated with disease activity parameters, while structural hip progression was associated with early onset of the disease, longer time to diagnosis, geographic origin, and JIA subtypes. Anti-TNF therapy was found to be effective in reducing structural damage progression.
MUSCULOSKELETAL CARE
(2023)
Article
Biochemistry & Molecular Biology
Polina Kamzeeva, Ivan Petushkov, Ekaterina Knizhnik, Robert Snoeck, Yuri Khodarovich, Ekaterina Ryabukhina, Vera Alferova, Artur Eshtukov-Shcheglov, Evgeny Belyaev, Julia Svetlova, Tatiana Vedekhina, Andrey Kulbachinskiy, Anna Varizhuk, Graciela Andrei, Andrey Aralov
Summary: Emerging and re-emerging viruses cause global outbreaks and epidemics, highlighting the need for new antiviral drugs. Researchers synthesized and evaluated nucleoside analogs and their derivatives, finding that one compound had antiviral activity against coronaviruses and specifically inhibited biocondensate formation important for replication.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Urology & Nephrology
Evert Cleenders, Priyanka Koshy, Elisabet Van Loon, Katrien Lagrou, Kurt Beuselinck, Graciela Andrei, Marta Crespo, Katrien De Vusser, Dirk Kuypers, Evelyne Lerut, Kris Mertens, Olga Mineeva-Sangwo, Parmjeet Randhawa, Aleksandar Senev, Robert Snoeck, Ben Sprangers, Claire Tinel, Amaryllis Van Craenenbroeck, Jan van den Brand, Marc Van Ranst, Geert Verbeke, Maarten Coemans, Maarten Naesens
Summary: This study aimed to investigate the value of performing a biopsy at the time of BKPyV-DNAemia in distinguishing presumptive and proven PyVAN. The results showed that plasma viral load had good predictive value for SV40 positivity, while the dichotomy of SV40 immunohistochemistry did not predict viral clearance. Therefore, the distinction between presumptive and proven PyVAN based on SV40 immunohistochemistry has limited clinical value.
KIDNEY INTERNATIONAL
(2023)
Article
Pediatrics
M. Wallimann, K. Bouayed, E. Cannizzaro, D. Kaiser, A. Belot, E. Merlin, S. Poignant, C. Wouters, F. Hofer, T. Saurenmann, A. Koryllou, R. Carlomagno, M. Mejbri, M. Hofer, K. Theodoropoulou
Summary: This study aims to identify predictors of disease evolution in patients with systemic juvenile idiopathic arthritis (sJIA). The results suggest that early disease onset, persistence of arthritis in the first year, and the use of synthetic DMARD may predict a persistent disease course.
PEDIATRIC RHEUMATOLOGY
(2023)
Article
Immunology
Luna Dillemans, Youri Bekhuis, Albrecht Betrains, Karen Yu, Maarten van Hemelen, Noemie Portner, Lien De Somer, Patrick Matthys, Jeroen Breckpot, Thomas Tousseyn, Marijke Peetermans, Paul Proost, Carine Wouters, Steven Vanderschueren
Summary: We report a fatal case of a 20-year-old woman with refractory adult-onset Still's disease accompanied by fulminant macrophage activation syndrome and atypical hemolytic uremic syndrome. This case highlights the importance of prompt genetic assessment of complement-encoding alleles, in addition to HLH-related genes, in patients with severe adult-onset Still's disease with recurrent macrophage activation syndrome and features of thrombotic microangiopathy.
CLINICAL IMMUNOLOGY
(2023)